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Background: Splenic cysts are uncommon and very rarely malignant therefore their treatment isn't standardized. In case of symptomatic cysts different surgical approaches have been suggested. Primary malignant lymphoma of the spleen comprises less than 1% of non-Hodgkin's lymphomas. To our knowledge, only 203 cases of splenic large B-cell lymphoma (LBCL) have been reported to date and only 2 of them were fibrin-associated splenic cysts., Case Presentation: 27-year-old model with a 19 × 13 cm splenic cyst without data of malignancy in the preliminary study and therefore treated with laparoscopic deroofing. After histological diagnosis of LBCL with a fibrin/EBV-associated splenic pseudocyst, the patient received 4 cycles of Rituximab and a laparoscopic splenectomy was performed due to resurgence of the pseudocyst. No evidence of malignancy has been found during follow up (EBV viral load every 3 months during the first year, PET-CT every 6 months during the first year and annual afterwards) performed after the splenectomy., Discussion and Conclusions: The value of tumor markers and radiology for diagnosis of splenic cysts is put into question. Only 60 cases of Fibrin-associated LBCL (FA-LBCL) have been described in the literature therefore there are no treatment guidelines for them even though surgery together with systemic treatment has been the prevalent route with good results in most cases., (© 2024. The Author(s).)

A 12-year-old domestic shorthair cat was presented with relapsing noncardiogenic chylothorax. CT-lymphangiography of the thorax confirmed bilateral pleural effusion without evidence of an underlying intra-thoracic origin. Abdominal CT-lymphangiography revealed a 2.6 cm diameter splenic mass surrounded by chylous effusion actively collecting during ultrasonographic assessment. Following splenectomy, histopathological analysis revealed that the splenic mass exhibited characteristics indicative of splenic angiosarcoma. This case report highlights the utility of advanced thoracic and abdominal imaging, notably CT-lymphangiography, in the diagnostic evaluation of chylothorax in cats. The identification of a splenic mass encased in chylous effusion should prompt a proactive case management strategy., (© 2024 American College of Veterinary Radiology.)

Objective: To explore the genetic basis and pathogenesis for a child with type I Hereditary hemorrhagic telangiectasia (HHTⅠ) and Splenic sinus shore cell hemangioma (LCA)., Methods: A child with HHT complicated with LCA diagnosed at the First Affiliated Hospital of Dali University in April 2022 was selected as the study subject. Clinical data of the child and her relatives were collected, and pathogenic variants were screened by whole exome sequencing. Candidate variant was verified by Sanger sequencing and bioinformatic analysis., Results: The patient, a 16-year-old female, had recurrent epitaxis since childhood, which sometimes necessitated hemostasis treatment. She also had splenectomy due to splenic rupture and was diagnosed with LCA. Her father and grandmother also had a history of recurrent epitaxis. Her father had deceased due to cerebral vascular rupture. The child was found to harbor a c.360+1G>A variant in the ENG gene. The same variant was not found in her asymptomatic mother and brother., Conclusion: The c.360+1G>A variant of the ENG gene probably underlay the pathogenesis in this child.

Objective: To evaluate the reliability of preoperative abdominal ultrasonography as a staging tool for dogs with hemoperitoneum due to presumed splenic tumor rupture, focusing on the detection of metastatic lesions in the liver., Animals: 99 dogs from 20 emergency and specialty hospitals across the US., Methods: Dogs with nontraumatic hemoperitoneum secondary to splenic tumor rupture were included. A post hoc analysis was conducted on data from a nationwide prospective trial investigating novel treatments for canine hemangiosarcoma. The accuracy of preoperative staging was assessed by comparing ultrasonographic findings with intraoperative observations and histologic findings., Results: On preoperative ultrasonography, there was a 20% incidence of liver lesions identified, with no association to liver lesions seen during operation. Notably, 22% of liver lesions observed during operation were missed on preoperative ultrasonography. The presence of liver lesions on preoperative ultrasonography was associated with a higher likelihood of a benign splenic tumor diagnosis. There was no association between the identification of liver lesions on preoperative ultrasonography and the presence of metastatic disease on liver biopsy, with a sensitivity and specificity of 19% and 82%, respectively. Additionally, ultrasound had low sensitivity in detecting intra-abdominal lesions beyond the liver and spleen, with 82% of these lesions missed preoperatively., Clinical Relevance: This study challenges conventional perceptions around the approach to staging in dogs with hemoperitoneum. These findings advocate for a reevaluation of the staging approach, with more comprehensive modalities like whole-body CT or MRI potentially being more warranted.

Competing Interests: Competing interests The authors declare that they have no competing interests.

A 48-year-old woman underwent transcatheter arterial embolization (TAE) for a splenic artery aneurysm, which resulted in a partial splenic infarction in the middle lobe. Five years after TAE, a 20-mm diameter mass in the noninfarcted area of the spleen was detected on imaging, which grew to 25 mm in diameter after 6 months. MRI after gadolinium administration showed a 35 × 34 mm mass within the superior pole and 15 × 12 mm mass within the inferior pole. The patient underwent laparoscopic splenectomy and had an uneventful postoperative recovery. No evidence of recurrence was observed during the 2-year follow-up period after surgery. The mass was pathologically confirmed to be sclerosing angiomatoid nodular transformation (SANT) of the spleen. While some studies hypothesize that SANT is a response to vascular injury or trauma, to the best of our knowledge, there have been no previous reports of SANT occurring after procedures directly affecting splenic blood flow. Additionally, multifocal SANTs are reported to be very rare, accounting for only 4.7% of all reported SANTs of the spleen. We highlight a rare course of SANT of the spleen and discuss the possible relationship between blood flow abnormalities and the appearance of SANT., (© 2023. Japanese Society of Gastroenterology.)

Background: Angiosarcomas are rare tumors that can be difficult to diagnose due to subtle changes in the vascular endothelium. When there is evidence to suggest malignancy, such as a pathologic fracture, further investigation is needed, and a high suspicion for angiosarcoma needs to be present so that appropriate immunohistochemical stains are utilized on biopsied tissue. In situations where such suspicion is high and prior biopsies have been negative, performance of splenectomy, can be both diagnostic and therapeutic when splenomegaly is present., Case Report: This is a case of a 52-year-old woman with splenomegaly, initially attributed to infection, in the setting of upper respiratory symptoms and thrombocytopenia. Three months later, however, she presented with back pain. Imaging showed lytic bone lesions with pathologic vertebral fracture and numerous liver lesions that were too small to characterize further. Initial biopsies of the liver and bone did not reveal a pathologic process. Several months later, still without a unifying diagnosis, she presented to our institution. MRI of the brain was done for neurologic concerns and showed pathologic enhancement in the calvarium. A PET scan showed diffuse avidity of the skeleton and spleen. After discussing the case with a hematologist, splenectomy was performed for both diagnostic and therapeutic purposes. Angiosarcoma was identified in the spleen and in a PET-directed bone biopsy. With a definitive diagnosis, she returned home and subsequently elected to pursue hospice care., Conclusion: When there is a high clinical suspicion for malignant angiosarcoma, a multidisciplinary approach is necessary to direct both tissue acquisition and necessary histochemical staining., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Bartonella species are gram-negative coccobacilli that are globally recognized as significant pathogens causing zoonotic infections. Among Bartonella species, B. henselae, B. quintana , and B. bacilliformis are prominent pathogens causing infections in humans, often manifesting as infective endocarditis. Bartonella endocarditis can pose diagnostic challenges due to its indolent presentation and limitations of standard microbiological culture techniques to identify the organism. We report a case of a 23-year-old male, who initially presented with the manifestations of hepatosplenic T-cell lymphoma, later diagnosed with blood culture-negative endocarditis caused by B. henselae . The patient had a complicated clinical course including pancytopenia, hepatosplenomegaly, and severe aortic valve regurgitation. Despite negative blood cultures, diagnostic clues included persistent fevers and bicuspid aortic valve with abscess. High Bartonella IgG titers (>1:800) supported the diagnosis. This case underscores the importance of considering Bartonella species in patients with suspected endocarditis, particularly in those with predisposing factors and negative blood cultures. Diagnosis relies heavily on serologic assays due to low sensitivity of conventional culture methods. Treatment involves a multidisciplinary approach with antibiotics and surgical intervention for optimal outcomes. Timely recognition and management are crucial to mitigate the high mortality associated with Bartonella endocarditis, and we hope this article offers insight for clinicians., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Atraumatic splenic rupture (ASR) is a rare occurrence but an important clinical entity. Although trauma is the most common cause of splenic rupture, there is limited literature on ASR. This case report discusses a 59-year-old woman presenting with tension hydrothorax and ASR in the setting of non-small cell lung carcinoma requiring emergent chest tube insertion and emergent splenectomy. Her hospital course was complicated by pulmonary embolism and thrombosis of the inferior vena cava. The patient expired three months after her initial presentation. This patient's presentation represents only the second documented case of atraumatic splenic rupture secondary to metastatic lung carcinoma without pathological evidence of splenic metastasis. Atraumatic splenic rupture secondary to metastatic NSCLC is a rare occurrence; though failure to detect, it may be fatal. Pathologic ASR may be an occult presentation of lung malignancy and in the presence of confirmed NSCLC may portend a poor prognosis.

Background: Littoral cell angioma (LCA) is a rare vascular primary tumor of the spleen with no more than 440 cases described so far. Although often seen as benign, it is described to have malignant potential and to be associated with other immunologic disorders or malignancies., Case Presentation: We present the case of LCA in a 75-year old man with a concomitant non-Hodgkin lymphoma and medical history of malign melanoma. The tumor was discovered incidentally after splenectomy for splenomegaly and refractory thrombocytopenia. The post-operative period was uneventful., Conclusion: Our case is the first to report an association of LCA with both lymphoma and melanoma thus far. It emphasizes the need for a thorough total body examination for synchronous diseases and close follow-up to reveal associated malignancies or immunologic disorders. Further research is required to identify etiologic and pathogenetic mechanisms behind this tumor and a common basis between the three diseases., Key Words: Littoral Cell Angioma, Neoplasm, Splenectomy, Solid Spleen Tumor.

Splenic rupture secondary to blunt trauma is a common condition. Non-traumatic, also known as spontaneous or pathological splenic rupture is an uncommon, but potentially life-threatening condition. Spontaneous splenic rupture caused by a primary splenic tumor is rare. In this case study, we present a special, benign tumor causing splenic rupture. Our 78-year-old female patient was hospitalized due to left shoulder pain and chest discomfort. Her blood pressure was low, the laboratory tests showed anemia, and the chest CT scan involving also the upper abdomen raised the suspicion of a splenic rupture. During the emergency splenectomy, there was a large amount of blood in the abdominal cavity. Macroscopic pathological examination of the removed spleen showed multifocal cystic lesions that led to splenic rupture. Immunhistochemical analyses revealed a littoral cell angioma. Littoral cell angioma is a rare, benign vascular tumor of the spleen, which is thought to originate from the red pulp sinuses lined with littoral cells. The aim of our report is to describe an unusual cause of sudden splenic rupture without traumatic history, the histologically benign littoral cell angioma that has not been published in Hungary. Orv Hetil. 2023; 164(10): 393-397.

Background: Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma. Patients usually present with splenomegaly and pancytopenia but without lymphadenopathy. Immunohistochemistry (IHC) staining of bone marrow biopsy shows intra-sinusoidal infiltration of CD3 and CD56 T-lymphocytes. Current treatment strategy of HSTCL includes a CHOP regimen (cyclophosphamide, adriamycine, vincristine, prednisone) followed by autologous transplantation., Case: A 28-year-old male presented with abdominal fullness, weight loss, and massive splenomegaly. Laboratory findings revealed pancytopenia. A CT scan of the abdomen displayed hepatomegaly and massive splenomegaly. The bone marrow pathology examination showed monotonous medium-sized lymphocytes with some cluster of atypical lymphocytes with loosely condensed chromatin and pale cytoplasm. The intra-sinusoidal location was more prominent after using IHC staining of CD3 and CD56, which are characteristics of HSTCL. We administered CHOP-based regiment every 3 weeks for 3 cycles; however, the response was a stable disease. Since the splenomegaly was still massive and compromised the patient, the multidisciplinary team decided to perform splenectomy. Unfortunately, the patient did not survive the surgery., Conclusion: Hepatosplenic T-cell lymphoma is a rare aggressive disease, which is part of peripheral T-cell lymphoma. CHOP-based chemotherapy appeared to be ineffective, and we need further studies to find the optimal treatment of HSTCL.

Prolonged fever with pancytopenia and hepatosplenomegaly is a clinical entity frequently encountered by physicians. The diagnosis of such cases is challenging due to the diversity of differential diagnoses. Hepatosplenic T-cell lymphoma is a rare and aggressive type of non-Hodgkin lymphoma that can present with massive hepatosplenomegaly, pancytopenia and prolonged fever. Most of the patients are young men and the majority are associated with chronic immunosuppression. We report a 40-year-old immunocompetent woman with prolonged fever and pancytopenia due to hepatosplenic T-cell lymphoma.

Objective: To evaluate the validity of the double two-thirds rule for a diagnosis of splenic hemangiosarcoma in dogs with nontraumatic hemoperitoneum due to a ruptured splenic mass., Sample: Systematic literature review., Procedures: 3 databases (PubMed, CAB abstracts, and World of Science) were searched in November 2020. Articles were included if data on dogs with nontraumatic hemoperitoneum due to a splenic mass were included and subsequent pathologic diagnosis could be determined., Results: In total, 2,390 unique articles were identified, with 66 articles meeting the criteria for full-text review and 14 articles included for analysis. A total of 1,150 dogs were evaluated, with 73.0% (840/1,150) of dogs being diagnosed with a malignant splenic lesion and 27.0% (310/1,150) being diagnosed with a benign splenic lesion. Of the malignancies, 87.3% (733/840) were hemangiosarcoma. Levels of evidence were low, and bias was high as most included studies were retrospective case series., Clinical Relevance: The double two-thirds rule should be refined when evaluating dogs with nontraumatic hemoperitoneum from a ruptured splenic mass, with more dogs being diagnosed with a malignancy and hemangiosarcoma specifically than the double two-thirds rule indicates. These findings may be useful in an emergency setting to guide owners on potential diagnoses for dogs with nontraumatic hemoperitoneum due to a ruptured splenic mass. However, there remains a portion of these dogs with benign conditions and nonhemangiosarcoma malignancies that may have a good long-term prognosis compared to dogs with hemangiosarcoma. Studies with higher levels of evidence, lower risks of bias, and large case numbers are needed in the literature.

We present the case of a 10-year-old boy who was admitted with 3 months of episodic febrile neutropenia and a new petechial rash. Routine bloods identified neutropenia, thrombocytopenia, and a raised alanine aminotransferase. The dermatology team was consulted in light of the symmetrical petechial eruption of the upper torso. A punch biopsy of the lesion was consistent with early capillaritis. The results showed superficial dermal red blood cell extravasation with mild perivascular lymphohistiocytic inflammatory infiltrate. There was no evidence of an atypical lymphoid infiltrate in the skin biopsy. An initial bone marrow aspirate showed an abnormal mature T-cell population consisting of CD4 and CD8 T cells with gamma-delta positivity. Karyotyping was also done, which demonstrated isochromosome 7q. These findings were consistent with a diagnosis of hepatosplenic T-cell lymphoma (HSTL). The patient underwent fourth-line chemotherapy due to refractory relapsing disease but sadly passed away within 12 months of diagnosis. HSTL is a rare and aggressive subset of peripheral T-cell lymphoma. Prognosis is poor with a median survival of <1 year from diagnosis. However, reports suggest improved outcomes if intensive, early, high-dose chemotherapy is used alongside hematopoietic stem cell transplantation. Therefore, there is an impetus to attain early diagnosis for aggressive early treatment and improved patient outcomes. Capillaritis, presenting as asymptomatic nonpalpable purpura, can be a rare presenting feature of HSTL. Dermatologists could play a pivotal role in the early recognition of this rare but aggressive hematological malignancy and promote prompt treatment resulting in better patient outcomes., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

The article presents a rare case of bone marrow metastasis of the spleen angiosarcoma. The observation is of particular interest due to the fact that secondary bone marrow damage in angiosarcoma in the vast majority of cases is due by primary tumor growth in the spleen. Clinically, such cases may resemble the course of blood diseases with hematological disorders and splenomegaly. Patients come into the field of view of a hematologist, and the final diagnosis is unexpected to the attending physician. Detection of angiosarcoma growth in a bone marrow trephine biopsy during morphological examination can be a rare finding for a pathologist. In this regard, the presented case is of interest not only for pathologists, but also for doctors of clinical specialties.

There is currently no evidence that a severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mRNA vaccine might be associated with the development of autoimmune hemolytic anemia or disease progression in patients with mature B-cell neoplasm. Our patient was a 71-year-old man with indolent mature B-cell neoplasm who had been monitored for many years without treatment. After receiving the second dose of the BNT162b2 mRNA COVID-19 vaccine, he developed severe warm autoimmune hemolytic anemia. Although steroid therapy improved his anemia, he continued to develop IgM-monoclonal gammopathy, renal insufficiency, and splenomegaly. He was diagnosed with splenic marginal zone lymphoma after undergoing splenectomy. The splenectomy improved the patient's symptoms. We assessed his SARS-CoV-2 specific antibody response, but the patient's serologic response to the vaccine was impaired. In patients with mature B-cell neoplasm, a non-specific immune response after vaccination might be associated with paraneoplastic syndromes.

Objective: To determine whether premature death occurred among dogs with nonmalignant splenic histopathologic findings after splenectomy for nontraumatic hemoabdomen., Animals: 197 dogs with nontraumatic hemoabdomen that underwent splenectomy and histopathologic evaluation between 2005 and 2018., Procedures: Information was obtained from electronic medical records, dog owners, and referring veterinarians to determine patient characteristics, histopathologic findings, survival information, and cause of death. Dogs were grouped based on histopathological diagnosis and outcome, and median survival times (MSTs) and risk factors for death were determined., Results: Histopathologic findings indicated malignancy in 144 of the 197 (73.1%) dogs with nontraumatic hemoabdomen. Hemangiosarcoma was diagnosed in 126 dogs (87.5% of those with malignancies and 64.0% of all dogs). Nine of 53 (17%) dogs with nonmalignant histopathologic findings had an adverse outcome and premature death, with an MST of 49 days. Risk factors for this outcome included low plasma total solids concentration, an elevated hemangiosarcoma likelihood prediction score, and a medium or high hemangiosarcoma likelihood prediction score category., Conclusions and Clinical Relevance: This study showed that there is a group of dogs with nontraumatic hemoabdomen due to splenic disease that have nonmalignant histopathologic findings after splenectomy, but nonetheless suffer an adverse outcome and die prematurely of a suspected malignancy. Further evaluation of potential at-risk populations may yield detection of otherwise overlooked malignancies.

BACKGROUND Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign disease of the splenic red pulp of unknown etiology. Definite diagnosis is made on histopathology alone as it has no diagnostic radiologic characteristics. CASE REPORT We present a case of a large incidental splenic mass in a middle-aged man, whose refractory anemia resolved with splenectomy. Our initial imaging studies failed to differentiate this lesion from other splenic lesions like hamartoma and haemangioma. To the best of our knowledge, no SANT cases in the literature have been investigated with red cell scan, as performed in our patient, nor has any case had resolved anemia after treatment. Splenectomy was offered as malignancy could not be safely excluded. Histopathology confirmed the diagnosis of SANT. The patient made good recovery and had no signs of recurrence 2 years later. CONCLUSIONS SANT of the spleen is a rare condition that can mimic many other splenic tumors and poses diagnostic challenge when histopathology is unavailable. Our case adds to the number of SANT reported in literature in the hope of elucidating the pathophysiology of this rare condition.

Background: The recent COVID-19 pandemic has raised concerns about patient diagnosis and follow-up of chronically ill patients. Patients suffering from chronic illnesses, concomitantly infected by SARS-CoV-2, globally tend to have a worse prognosis and poor outcomes. Renal tropism and acute kidney injury following SARS-CoV-2 infection has recently been described in the literature, with elevated mortality rates. Furthermore, patients with pre-existing chronic kidney disease, infected by SARS-CoV-2, should be monitored carefully. Here, we report the case of a 69-year-old patient with splenic marginal zone lymphoma, suffering from longstanding chronic kidney disease following SARS-CoV-2 infection., Case Presentation: A 69-year-old male patient previously diagnosed with pulmonary embolism and splenic marginal zone lymphoma (Splenomegaly, Matutes 2/5, CD5 negative and CD23 positive), was admitted to the hospital with shortness of breath, fever and asthenia. A nasopharyngeal swab test was performed in addition to a CT-scan, which confirmed SARS-CoV-2 infection. Blood creatinine increased following SARS-CoV-2 infection at 130 μmol/l, with usual values at 95 μmol/l. The patient was discharged at home with rest and symptomatic medical treatment (paracetamol and hydration), then readmitted to the hospital in August 2020. A kidney biopsy was therefore conducted as blood creatinine levels were abnormally elevated. Immunodetection performed in a renal biopsy specimen confirmed co-localization of SARS-CoV2 nucleocapsid and protease 3C proteins with ACE2, Lewis x and sialyl-Lewis x antigens in proximal convoluted tubules and podocytes. Co-localization of structural and non-structural viral proteins clearly demonstrated viral replication in proximal convoluted tubules in this chronically ill patient. Additionally, we observed the co-localization of sialyl-Lewis x and ACE2 receptors in the same proximal convoluted tubules. Reverse Transcriptase-Polymerase Chain Reaction test performed on the kidney biopsy was negative, with very low Ct levels (above 40). The patient was finally readmitted to the haematology department for initiation of chemotherapy, including CHOP protocol and Rituximab., Conclusions: Our case emphasizes on the importance of monitoring kidney function in immunosuppressed patients and patients suffering from cancer following SARS-CoV-2 infection, through histological screening. Further studies will be required to decipher the mechanisms underlying chronic kidney disease and the putative role of sialyl-Lewis x and HBGA during SARS-CoV-2 infection., (© 2021. The Author(s).)

We report the case of a patient diagnosed with a splenic marginal zone lymphoma with a simultaneous finding of hepatitis B virus infection, who responded to antiviral treatment and splenectomy. We highlighted this association described in the literature and its possible causal role, as well as the available therapeutic choices., Competing Interests: Declaration of Competing Interest There are no conflicts of interest to disclose., (Copyright © 2019 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. All rights reserved.)

A 12-year-old neutered male German shepherd dog was evaluated after dying suddenly at home. A few hours prior to the unexpected death the dog displayed anorexia and lethargy. Post-mortem examination and histopathology led to a diagnosis of marked hemoabdomen and hypovolemia due to a single ruptured splenic hemangiosarcoma., (Copyright and/or publishing rights held by the Canadian Veterinary Medical Association.)

Splenic angiosarcoma (SA) is a rare disease that can cause spontaneous splenic rupture leading to unexpected death. The rare incidence and non-specific clinical presentations made the early correct diagnosis of SA impossible in clinical practice. Even with medical intervention, 80% of patients died within 6 months after diagnosis. Here, we report a man who had complained of abdominal distension for 2 weeks and succumbed to the disease nine hours after admission. Diagnosis of hepatic and splenic angiosarcomas was based on post-mortem histopathological examination and immunohistochemistry, and the cause of death was hemorrhagic shock caused by spontaneous splenic rupture secondary to SA. This present case was the first forensic autopsy of spontaneous splenic rupture secondary to SA, which can highlight the diagnosis of rare diseases in forensic practice, and forensic pathologists should bear in mind these rare diseases even in routine practice., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell neoplasm that most commonly arises from a small subset of γ/δ T-cell receptor-expressing lymphocytes. HSTCL is more common in adolescent and young adults and has a rapidly progressive clinical course and poor outcome due to its refractoriness to conventional chemotherapy regimens. Approximately 20% of the cases arise in the background of chronic immunosuppression or immune dysregulation. Patients commonly present with constitutional symptoms, hepatic and liver enlargement, and cytopenias; hematophagocytic syndrome can also occur. The most frequent chromosomal aberrations associated with HSTCL are isochromosome 7q and trisomy 8, and most cases harbor mutations in genes involved in chromatin modification or the JAK/STAT pathway. The rarity of this disease, along with lack of nodal involvement and presenting symptoms that mimic different entities including infectious etiologies, makes this lymphoma a significant diagnostic challenge. In this review, we highlight the clinical and pathologic features of HSTCL. Moreover, we summarize the results of recent molecular studies suggesting potential targets for novel therapeutics strategies., (© 2020 by The American Society of Hematology.)

A 75-year-old woman presented to our hospital with a history of fever, cervical lymphadenopathy, and fatigue. Computed tomography(CT)revealed systemic lymphadenopathy with prominent splenomegaly. Axillary lymph node biopsy results revealed diffuse proliferation of atypical lymphoid cells with arborizing high endothelial venules. Immunohistochemical staining was positive for CD3, CD5, and CD10, but negative for CD20 and CD79a. Given these findings, a diagnosis of angioimmunoblastic T-cell lymphoma(AITL)was made. Due to the extremely high tumor burden, pre-therapy with corticosteroids was initiated. However, the patient suddenly went into hemorrhagic shock. Contrast-enhanced CT revealed abdominal bleeding due to splenic rupture. Bleeding was rapidly controlled using transcatheter arterial embolization(TAE). Five days after TAE, mini-CHOP therapy was initiated. Splenomegaly is common in hematologic disease. Owing to the lethality of the condition, in cases of progressive anemia with splenomegaly in patients with hematologic disease, the possibility of splenic rupture should be considered. Since TAE carries no risk of post-splenectomy infection and allows timely resumption of chemotherapy, it could be considered as one of the preferred treatment choices for splenic rupture in hemodynamically unstable patients.

Background: Splenic cord capillary hemangioma is a rare benign vascular lesion classified as a splenic hamartoma. On the other hand, non-islet cell tumor hypoglycemia (NICTH) is one of the rare causes of spontaneous hypoglycemia and is considered to be one of the paraneoplastic syndromes. To the best of our knowledge, this is the first reported case of a splenic cord capillary hemangioma with NICTH., Case Presentation: A 25-year-old male was referred to our hospital with hypoglycemia. Except for his low blood sugar, there were no abnormal findings from laboratory tests, which included an endocrinological examination. Enhanced computed tomography confirmed the presence of a solid mass measuring about 6 cm in the retroperitoneum, and a tumorectomy was performed. During this operation, it became clear that the tumor turned out to be a splenic parenchyma, and as a result, a total splenectomy was performed. Microscopically, we diagnosed this as a cord capillary hemangioma, and through immunohistochemistry, we found that some tumor cells were positive for insulin-like growth factor -II. Fortunately, the hypoglycemia-related symptoms disappeared after surgical resection was performed. The patient is still alive and well without evidence of local tumor recurrence 15 years after the operation., Conclusions: Splenic cord capillary hemangioma, one of the types of splenic hamartomas, is a very rare benign vascular lesion and might be associated with hypoglycemia thought to be NICTH.

Mucinous cystadenoma are rare benign mucin-producing tumours. Primary mucinous neoplasms most commonly involve ovary, pancreas or appendix. They may rupture to produce mucous ascites/pseudomyxoma peritonei. We are describing a 51-year-old man who presented to us with symptomatic gallstone disease but during preoperative ultrasound was found to have cystic lesions in spleen with free fluid in the abdomen. Splenectomy and drainage of mucin were done. Histopathology was done and a final diagnosis of primary mucinous cystadenoma of spleen with mucinous ascites was made., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Spontaneous splenic rupture (SSR) is a rare but potentially life-threatening entity. It can be due to neoplastic, infectious, haematological, inflammatory and metabolic causes. An iatrogenic or an idiopathic aetiology should also be considered. Depending on the degree of splenic injury and the haemodynamic status of the patient, it can be managed conservatively. A 61-year-old man presented to the emergency department with an acute abdomen, hypovolaemic shock and clotting abnormalities. However, his focused assessment with sonography for trauma showed no evidence of an aortic aneurysm, rupture or dissection. Further investigation with a CT angiogram aorta confirmed a subcapsular splenic haematoma with free fluid in the pelvis and a mass in the superior pole of the spleen. He was diagnosed with an SSR. He was initially managed non-operatively. However, his repeat CT showed an enlarging haematoma and he underwent embolisation of his splenic artery. Ultrasound-guided core biopsy of his splenic mass confirmed the diagnosis of diffuse large B-cell lymphoma. This paper will discuss the clinical presentation, differential diagnosis and management of SSR. Furthermore, it provides an important clinical lesson to maintain a high index of clinical suspicion for splenic injury in patients presenting with left upper quadrant abdominal pain radiating to the shoulder. This case also reinforces the importance of close observation and monitoring of those individuals treated conservatively for signs of clinical deterioration., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

We report a case of splenic marginal zone B-cell lymphoma discovered in a 73-year-old man with cold agglutinin disease. PET/CT revealed splenomegaly with focally intense uptake of F-FDG and diffusely increased bone marrow uptake, which was considered to be secondary to hemolytic anemia. Splenectomy was performed. The histopathology of the spleen showed splenic marginal zone B-cell lymphoma with partial splenic infarction, which correlated with the area of focal intense FDG uptake. Depending on the time since onset, splenic infarctions can present as focal FDG accumulation.

A 59-year-oldwoman presented with a 2-month history of malaise, abdominal distention and unintentional weight loss. She was initially managed as community acquired pneumonia with a suspicion of underlying chronic liver disease but she deteriorated rapidly into a multiorgan failure necessitating transfer to intensive care unit of a tertiary hospital. She was investigated with liver and bone marrow biopsy that confirmed the diagnosis of hepatosplenic T cell lymphoma. She was treated with cyclophosphamide, doxorubicin, vincristine, etoposide and prednisolone chemotherapy that was changed to salvage ifosfamide carboplatin etoposide (ICE) chemotherapy due to poor response with first-line chemotherapy and disease progression. Unfortunately, her disease progressed further and she opted for palliative management., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Rationale: Littoral cell angioma (LCA) is a rare primary vascular neoplasm of the spleen. It can be benign or malignant. Pathology and immunohistochemistry are the gold standards for the diagnosis of LCA. Therefore, splenectomy is recommended for the purpose of diagnosis and treatment, and subsequent follow-up is necessary. There are limited reports about LCA. Here, we present a case of a female patient with LCA undergoing laparoscopic splenectomy in order to provide clinical experience in LCA treatment., Patient Concerns: A 32-year-old female attended the outpatient Department of Hepatobiliary Surgery for follow-up of hepatic hemangiomas. The patient presented with intermittent abdominal distension, which was slightly under no obvious inducement., Diagnosis: Physical examination found no signs of abdominal tenderness and rebound tenderness, and liver and spleen were impalpable. The contrast-enhanced computed tomography (CT) showed multiple space-occupying lesions in the spleen, mottled low-density lesions, multiple hypoattenuating nodules with no contrast enhancement on the arterious phase. Delayed contrast-enhanced helical CT scan displayed incomplete filling of hypodense splenic lesions., Interventions: Given that it was uncertain whether it was a benign or a malignant tumor, a laparoscopic total splenectomy was performed., Outcomes: The final pathological diagnosis was LCA. Her postsurgical course was uneventful, and no surgery-related complications were found. No signs of recurrence were observed in the 16 months after the operation., Lessons: LCA was a rare primary vascular neoplasm of the spleen, and laparoscopic splenectomy for LCA was safe and feasible, and postoperative course was uneventful. However, regular follow-up and long-time monitoring after splenectomy for LCA is recommended because of its potential malignant biological behavior.

We report a 48-year-old woman with metastatic infiltrating lobular carcinoma of the breast. Though her metastatic disease remained stable, she was repeatedly admitted for symptomatic anaemia and treated by red blood cell and platelet transfusions with increasing frequency as time elapsed. Abdominal examination and ultrasound revealed splenomegaly (27 cm span). A bone marrow biopsy showed fibrosis and foci of metastatic carcinoma. Splenectomy ameliorated her transfusion-dependent anaemia and thrombocytopaenia. Histopathology revealed multiple foci of metastatic carcinoma and scattered foci of extramedullary haematopoiesis. Differential diagnosis of anaemia and thrombocytopaenia in patients with cancer include bone morrow involvement by cancer cells, iron-deficiency anaemia, microangiopathies and chemotherapy suppression of haematopoiesis. Splenic involvement with cancer is common in patients with multivisceral disease. Many may regard transfusion-dependent severe anaemia and thrombocytopaenia as an end-stage disease in these patients. Nevertheless, palliative splenectomy should be considered in patients with possible hypersplenism who will otherwise survive for a relatively prolonged period of time., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

The hemophagocytic syndrome is a serious clinical-histological entity secondary to different diseases. Collapsing glomerulonephritis is a proliferative podocytopathy that usually has an unfavorable renal prognosis. We present a case in which both entities were associated, which is an infrequent form of hepatosplenic T-cell lymphoma. In addition, we review the role of the markers of podocyte dedifferentiation in this glomerulopathy and its pathophysiology and treatment.

Hepatosplenic T cell lymphoma (HSTCL) is a rare subtype of peripheral non-Hodgkin lymphoma, of which 20% of cases are associated with chronic immunosuppression. It often occurs in patients with inflammatory bowel disease treated with immunomodulating medications such a thiopurines or TNF-alpha inhibitors. Cytopenias are commonly seen but autoimmune hemolytic anemia (AIHA) is rare. Here we present a young male with longstanding ulcerative colitis on chronic azathioprine, exhibiting several rare features of HSTCL. He initially presented with refractory AIHA, thrombocytopenia, and massive splenomegaly, requiring splenectomy. Histologic examination of his spleen confirmed diagnosis of HSTCL. Approximately 3 months after diagnosis, he was found to have leukemic transformation, representing a secondary malignancy.

Background: Malignant fibrous histiocytoma has been uncommonly described in dogs. Several extranasal neoplasias have been reported to result hypertensive epistaxis. There are, however, no published case reports of extranasal malignant fibrous histiocytoma with concurrent hypertension and epistaxis in dogs., Case Presentation: A 10-year-old dog presented with a spontaneous massive epistaxis persisting for 5 days. The dog exhibited unstable hypertension, which was considered as a cause of epistaxis. The complete blood count, prothrombin time, and activated partial thromboplastin time were within the reference limits, and other systemic examination showed no abnormalities except for a splenic mass occupying more than one third of the abdomen. Histologic examination of the resected spleen revealed the characteristic features of a malignant fibrous histiocytoma. One week after splenectomy, the hypertension and epistaxis resolved clinically and did not recur on the 5-month follow-up., Conclusions: The dog's blood pressure and epistaxis normalized after malignant fibrous histiocytoma resection suggesting that hypertensive epistaxis may be a rare manifestation of canine malignant fibrous histiocytoma.