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Constrictive pericarditis can be a systemic manifestation of immune-mediated diseases. A timely diagnosis followed by ethiologic workup is essential for improving patient prognosis.

Background: Brentuximab Vedotin (BV) has revolutionized the treatment landscape for Hodgkin's lymphoma, yet its effects on pre-existing autoimmune disorders remain elusive. Methods: Here, we present four cases of patients with concurrent autoimmune conditions—Crohn's disease, vitiligo, type I diabetes, and minimal change disease—undergoing BV therapy for Hodgkin's lymphoma. The patients were treated with A-AVD instead of ABVD due to advanced-stage disease with high IPI scores. Results: Our findings reveal the surprising and complex interplay between BV exposure and autoimmune manifestations, highlighting the need for multidisciplinary collaboration in patient management. Notably, the exacerbation of autoimmune symptoms was observed in the first three cases where T-cell-mediated autoimmunity predominated. Additionally, BV exposure precipitated autoimmune thrombocytopenia in the vitiligo patient, underscoring the profound disruptions in immune regulation. Conversely, in the minimal change disease case, a disease characterized by a blend of B- and T-cell-mediated immunity, the outcome was favorable. Conclusions: This paper underscores the critical importance of vigilance toward autoimmune flare-ups induced by BV in patients with concurrent autoimmune conditions, offering insights for tailored patient care. [ABSTRACT FROM AUTHOR]

In the 2016 revision of the World Health Organization classification the term therapy-related myeloid neoplasia (t-MN) defines a subgroup of acute myeloid leukemia (AML) comprising patients who develop myelodysplastic syndrome (MDS-t) or acute myeloid leukemia (AML-t) after treatment with cytotoxic and/or radiation therapy for various malignancies or autoimmune disorders. We report the case of a 36 year old patient with t-MN (t-MDS) after achieving complete remission (CR) of a PML-RARA positive acute promyelocytic leukemia (APL) at 32 months after diagnosis. Initially classified as low risk APL and treated according to the AIDA protocol - induction and 3 consolidation cycles - the patient achieved a complete molecular response in September 2013 and started maintenance therapy. On follow-up PML-RARA transcript remained negative. In January 2016 leukopenia and thrombocytopenia developed and a peripheral blood smear revealed hypogranular and agranular neutrophils. Immunophenotyping in the bone marrow aspirate identified undifferentiated blast cells that did not express cytoplasmic myeloperoxidase. The cytogenetic study showed normal karyotype. The molecular biology tests not identified PMLRARA transcript. A diagnosis of t-MDS (AREB-2 - WHO 2008) was established. Treatment of AML was started with 2 “3+7” regimens and 1 MEC cycle. Two months from diagnosis, while in CR, an allogeneic HSCT from an unrelated HLA compatible donor was performed after myeloablative regimen. An unfavorable clinical evolution was followed by death on day 9 after transplantation. The occurrence of t-MNs during CR of APL represents a particular problem in terms of follow-up and differential diagnosis of relapse and constitutes a dramatic complication for a disease with a favorable prognosis.

Hodgkin lymphoma (HL) has become one of the most curable hematological neoplasia. Clinical and biological factors remain the main pillars guiding therapeutic strategies in HL. Recent studies have improved our understanding of the phenotype, the characteristics of histogenesis, and other possible mechanisms of lymphomagenesis, including the role of Epstein-Barr virus (EBV) infection. Tumor cells manipulate the microenvironment, allowing them to develop their malignant phenotype and evade the attack of the host's immune response so that the interaction between tumor cells and the reactive microenvironment determines not only the histological features but also the clinical-pathological characteristics and prognosis of these patients - essential for the development of future therapies targeting various other cellular components of the tumor microenvironment. This article aimed to evaluate the characteristics of the tumor microenvironment and malignant cells using histopathology and immunohistochemistry (IHC) techniques to highlight the association of EBV and to study the expression of characteristic antigens in malignant and non-malignant cells within the tumor mass (overexpression of BCL2 (B-cell lymphoma 2) in malignant cells, presence of PD1 (Programmed cell death Protein 1) on T lymphocytes, CD68+ macrophages in the tumor microenvironment, and presence of EGFR (epidermal growth factor receptor). The analysis of the data collected in this paper highlights several key parameters with prognostic value and statistical significance: the EBV infection at diagnosis, its association with low-intensity BCL2(+), the presence of CD68 with rosette formation, and the identification of specific vascularization patterns. The development of prognostic systems that take into account the integration of biological prognostic markers seems essential for a better risk stratification. [ABSTRACT FROM AUTHOR]

Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder that is characterized by the presence of an absolute monocytosis (1 × 10^ 9/l) in the peripheral blood, the overlap of myelodisplastic aspects and myeloproliferative aspects in the bone marrow and tendency to transform into acute myeloid leukemia. CMML is considered to be the most aggressive chronic myeloid leukemia. We present the case of a 48 years old woman who was hospitalized in March 2013 in the Center of Hematology and Bone Marrow Transplantation for anemia related symptoms. Initial investigations showed anemia, relative monocytosis (10% monocytes of the WBC differential) with an increasing absolute number of monocytes (> 1,000/μl) in the following months. Initial exploration of the bone marrow (aspirate and bone marrow biopsy and immunohistochemistry IHC tests) revealed elements of trilinear dysplasia and an increased percentage of myeloblasts (11-14%). In the next four months myeloblasts percentage remained below 20% (8-14%) and it has been observed a gradually increasing of monocytoid elements (> 20%). Immunophenotyping in the bone marrow aspirate identified a monocytic proliferation with high percentage (8%) of immature cells. The karyotype reported the presence of clones with t (1;3). Initially diagnosed as RAEB-2 (WHO) the case was recomitted in CMML-type 2 with a progression to acute myeloid leukemia (AML). Allogeneic hematopoietic stem cell transplantation (allo-HSCT) has been performed after getting the best possible therapeutic response with AML chemotherapy type (complete remission). Allo-HSCT was performed using myeloablative conditioning, 12 months after diagnosis. The patient is now in complete remission, 24 months after allo-HSCT.

We present the case of a 76 year old female patient admitted in the Department of Cardiology for physical asthenia, profuse sweating and dyspnea with orthopnea for about one month. Clinical and paraclinical assessments performed at admission confirmed the diagnosis of cardiac tamponade. Surgical intervention was performed and 400 mL of clear effusion were drained. Post-operative evolution was marked by recurrence of symptoms, requiring after 3 weeks a new drainage of 600 mL of clear effusion, and biopsy of the pericardium was performed. Pathological exam described serous pericarditis with chronic inflammatory infiltrate, xanthogranulomatous reaction intricated in the pericardium and mesothelial hyperplasia. The patient was subsequently transferred to the Department of Internal Medicine for further investigations. Physical examination showed a patient with altered general status, pallor, vesicular murmur absent in both bases, presenting cutaneous hyperpigmentation at the level of the right hemi-abdomen and hip with posterior extension, and a peripheral indurated erythematous plaque. The patient presented nodular masses of 3 cm in the right latero-cervical and bilateral axillary regions, non-adherent to the superficial structures, as well as adenopathic blocks in both inguinal regions. CT scan of the thorax and abdomen showed moderate bilateral pleuresia, minimal pericardial effusion (15 mm) and multiple adenopathies on both sides of the diaphragm. Skin biopsy was performed, as well as bone marrow aspirate and excision of a right axillary lymph node. Pathological exams and immunohistochemistry tests confirmed the diagnosis of Plasma Cells Castleman disease.

Scopul acestui studiu, parte a proiectului PERSOTHER, este implementarea unei tehnici noi - hibridizarea in situ cu imunofluorescenţa (FISH) - in diagnosticul limfoamelor Burkitt (BL), pentru prima dată în ţara noastră. BL este un limfom cu celulă B de mare agresivitate clinică. Sunt recunoscute 3 variante de LB: endemică (în Africa ecuatorială), sporadică (peste tot in lume) şi asociată cu imunodeficienţa. Clasificarea Organizaţiei Mondiale a Sănătăţii din 2008 a descris o nouă formă de limfom cu celulă B, neclasificabil, cu caractere intermediare între un limfom difuz cu celulă mare B şi un BL. Deoarece tratamentul BL este foarte agresiv, cu chimioterapie in doze mari, un diagnostic corect este obligatoriu. Citologia, morfologia şi imunofenotipul (CD20+, CD10+, BCL6+, BCL2-, Ki67 98-100%) sunt tipice pentru BL. Majoritatea au o translocaţie a genei MYC, localizate pe 8q24 cu gena pentru IgH, 14q32. Demonstrarea transocaţiei MYC este necesară pentru diagnosticul de BL şi pentru diferenţierea de un DLBCL, în unele cazuri. 22 de cazuri de BL au fost evaluate prin FISH pentru translocaţia MYC. 17 cazuri au fost pozitive, un caz a fost negativ şi 4 cazuri au fost neconcludente. Aceste rezultate sunt concordante cu literatura de specialitate: autorii raportează aproximativ 10% cazuri de BL fără transocaţie MYC. În aceste cazuri pot fi implicate alterări ale micro-ARN. Studiul actual evidenţiază importanţa FISH in diagnosticul BL, identifică unele dificultăţi tehnice ale metodei şi reprezintă pentru viitor, o bază a diagnosticului de rutină, în cazuri selecţionate.

Introduction: Essential thrombocythemia is a chronic myeloproliferative neoplasm associated with thrombo-hemorrhagic events and the progression to myelofibrosis or acute myeloid leukemia. The purpose of this article is to present real-world data on ET cases diagnosed and managed between 1998 and 2020 in the largest, tertiary hematology reference center in Romania and to evaluate the impact of thrombotic events on survival. Methods: A real-world, retrospective cohort-type study was conducted. We collected and statistically analyzed data from 168 patients who met the 2016 WHO diagnostic criteria for ET and who were managed between 1998 and 2020 in our center. Results: The median age at diagnosis of ET was 51.8 years, with a female predominance (66.07%). The JAK2V617F mutation was detected in 60.71% of patients. Leukocytosis at diagnosis was associated with a higher risk of thrombosis, and JAK2V617F-positive cases exhibited a 1.5-fold higher risk of developing thrombotic events. The average survival in ET with major thrombosis was 14.5 years versus 20.6 years in ET cases without major thrombosis. Other predictors of survival were high-risk IPSET score and age >60 years. Conclusions: Romanian patients diagnosed with ET are generally younger than 60 years and are predominantly female. The occurrence of thrombotic events was influenced by gender, leukocyte count at diagnosis and JAK2V617F positivity. Survival was impacted by age, the presence of JAK2V617F mutation, hypertension, major thrombotic complications and IPSET score. Notably, these findings warrant careful interpretation and further confirmation in the setting of prospective studies. [ABSTRACT FROM AUTHOR]

Background: During the last two years, the COVID-19 pandemic led to millions of disease-related deaths worldwide. The efforts of the scientific community facing this global challenge resulted in outstanding achievements. Thus, within one year, new mRNA-based vaccines against SARS-CoV-2 viral infection were released, providing highly efficient protection and showing a very good safety profile in the general population. However, clinical data collection after vaccination is a continuous process for the long-term safety of any new medical product. The aim of our paper is to present two cases of hematological malignancies: diffuse large B-cell non-Hodgkin lymphoma and T/NK-cell lymphoma, diagnosed shortly after the administration of the mRNA COVID-19 vaccine. Methods and Results: Case 1: A female patient was admitted with a suspicious cervical mass that emerged within one week after the administration of second dose of the BNT162b2 COVID-19 vaccine. Surgical removal followed by pathology assessment of the specimen confirmed the diagnosis of diffuse large B-cell non-Hodgkin lymphoma. Case 2: A male patient was admitted with multiple ulcerative oral lesions arising on the third day after the initial dose of the BNT162b2 COVID-19 vaccine. These lesions had a progressive character and during the following months were complicated with repetitive episodes of heavy oral bleeding, requiring blood transfusions. The incisional biopsy of the lesions and pathological assessment of the specimens confirmed the diagnosis of T/NK-cell lymphoma. Conclusions: The safety profile of the mRNA-based vaccines is an undeniable fact. In most cases, suspicions of potentially aggressive side effects were ruled out, proving to be transient post-vaccine reactions. Clinicians should remain alert to report any potentially aggressive manifestations emerging in the context of mRNA COVID-19 vaccination, such as these cases of hematological malignancies, in order to promote additional investigations on the particular mechanisms of action of COVID-19 vaccines and to provide the best medical care to the patients. [ABSTRACT FROM AUTHOR]

We report the case of a 79‐year‐old female patient previously treated for multiple myeloma that was referred to our hospital due to a growing painless right arm tumor. Imaging and pathology results confirmed the diagnosis of extramedullary plasmacytoma. The patient underwent external beam radiotherapy with complete clinical response at follow‐up. [ABSTRACT FROM AUTHOR]

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Copyright of Oncolog-Hematolog is the property of MEDICHUB MEDIA, S.R.L. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

POEMS syndrome (acronym consisting of: polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is an uncommon disorder associated with an underlying plasma cell dyscrasia. There is no single specific test for POEMS, and due to its rarity and heterogeneity, patients are often mis- or underdiagnosed. Castleman disease (CD) is a rare lymphoproliferative disorder, closely related to POEMS syndrome; ~11%–30% of POEMS patients are associated with concomitant CD. In contrast to frequently published reports on vascular events in POEMS syndrome affecting coronary arteries or lower limbs, cases of cerebrovascular events are rarely mentioned in literature. We hereby report a patient with POEMS syndrome accompanied by CD who presented recurrent strokes and splenic infarction. [ABSTRACT FROM AUTHOR]

Kikuchi–Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare disease that is frequently underdiagnosed due to clinical features that are similar to those of non-Hodgkin lymphomas, systemic lupus erythematosus (SLE), or infectious reactive lymphadenopathy. An excisional biopsy is required. We report a young Caucasian female diagnosed with KFD with skin lesions, complicating with SLE. The clinical course, laboratory, and CT findings are described, as are histopathologic features, for a better recognition of this rare disorder in clinical practice. [ABSTRACT FROM AUTHOR]

Objective: Primary bilateral dumbbell-shaped lumbar non-Hodgkin lymphomas with epidural and extraspinal involvement, are rare occurrences. Patients presenting at advanced stages and rapid evolution towards neurological impairment lead to diagnostic dilemmas for which only immunohistochemistry can provide a correct, although delayed solution. Case report: We report the first case of a bilateral, dumbbellshaped, lumbar lymphoma in a 65-year-old man with a medical history of chronic viral hepatitis type B and D under interferon treatment. The patient presented with back pain radiating down the right leg, with rapid progression to paraplegia and sphincter dysfunction. CT and MRI revealed a large dumbbell mass (approx. 5/5/10 cm) in the right paraspinal musculature, at the L4-L5 level, with intraspinal epidural extension. A similar mass of smaller size was described on the left side, almost mirroring the first lesion, the imagistic aspect suggesting a neural sheath tumor. Intraoperatively, in the right lumbar paraspinal musculature, a soft, yellowish region was discovered, the macroscopic appearance being rather suggestive for a diffuse infection. Clinical, imagistic and surgical findings were not conclusive, nor was the histological examination in light microscopy of the surgical specimen or of the bone marrow biopsy. Immunohistochemistry identified the presence of large B cells, leading to the diagnosis of B cell lymphoma. Although the patient was treated with systemic chemotherapy, his condition rapidly deteriorated and he died within 3 months. Conclusions: In the case of a lumbosacral, dumbbell shaped mass, developed both epidural and extraspinal, the differential diagnosis must include lymphoma. The histological examination, especially immunohistochemistry provided the final diagnosis. Delays in establishing a diagnosis, associated with a malignant evolution of lymphoma, diminish the chances of determining and applying a treatment strategy that could prolong survival. [ABSTRACT FROM AUTHOR]

Lymphomas represents neoplasms of the lymph reticular system (including Peyer's patches) and the gastrointestinal tract is the most commonly involved extranodal site. Lymphoma's incidence rised in past four decades so in this moment all extranodal lymphomas represent 30-40% from all Non-Hodgkin lymphomas. The stomach is the common site of GIT lymphomas (>85%) represented by mucosa associated lymphoid tissue (MALT), a indolent disease and diffuse large B-cell Lymphoma (DCBCL), a type of aggressive malignancy. Follicular lymphoma (FL), mantle cell lymphoma (MCL), small lymphocytic lymphoma (SLL), T-cell lymphoma (TCL) and plasmacytoma are very rare in the stomach. Lyphomas are localized on small intestine because it contains a lot of mucosal lymphoid tissue represented both by B- and T-cells such that immunocompromised patients can have reactive lymphohistiocytic infiltrates secondary to infections that can occur or mimic Hodgkin lymphoma. Burkitt lymphomas are often found on children and DLBC lymphomas are the commonest on adults. [ABSTRACT FROM AUTHOR]

Diffuse large B cell lymphoma (Diffuse Large B-Cell Lymphoma = DLBCL), the most common type of non-Hodgkin lymphoma (NHL) is characterized by a great heterogeneity in terms clinico-evolutionary and molecular histology. We present results of a study of 98 patients diagnosed and treated for DLBCL. Two thirds of patients from urban areas (61.2%), with a predominance of males (M = 63.4%, F = 36.6%), with a ratio M/ F = 2/1. Median age was 61.5 years, half of the subjects with ages between 48.75 and 71.15.3% of patients in the study group at diagnosis associated viral infections, most type hepatitis (HCV- 46.7%, HBV- 33.3%). Extranodal determinations were highlighted in a third of patients, the most common being the bone marrow, the circle Waldayer, stomach, pleuro-pulmonary, soft tissue, bone, brain, etc.. Factors that adversely influenced survival were advanced clinical stage at diagnosis (median survival reached after 54 months compared to 82 months in cases diagnosed in the early stages, this symptom B (correlated with disease relapse P = 0.058), presence of extranodal determinations (median survival of 25 months compared with 33 months in their absence), high levels of serum LDH (P = 0.010), Ki67 proliferation index > 70% (correlated with elevated LDH, P = 0.05) and phenotype of non-germinal center proliferation of tumor (median survival was reached after 48 months compared with 86 months in cases of DLBCL with germinal center phenotype). Association of monoclonal antibodies to PCT significantly improved (P = 0.01) obtaining RC study group. NHL DLBCL remains a group characterized by a great diversity aspect that determines the need to identify new prognostic factors and impact of new therapeutic targets. [ABSTRACT FROM AUTHOR]

The aim of this study is to analyze a group of patients with chronic lymphoproliferative disorders associated with B, C, D hepatitis viral infection. This group of chronic lymphoproliferative disordered patients with associated hepatitis viral infection has been diagnosed and monitored in the Hematology Department of the University Emergency Hospital of Bucharest, between December 2007 and January 2009. Our study is meant to observe the influence of the viral infection on clinical and biological evolution of the enrolled patients. The diagnosis of the chronic lymphoproliferative disorder was based on the bone marrow / lymph node biopsy and flow-cytometry analysis. The positive diagnosis for hepatitis viral infection was established by ELISA serological tests and viremia was performed by TaqMan method at INBI "Matei Bals" Bucharest. The analyzed group is made up of 41 patients, 25/41 (60,97%) females and 16/41 (39,02%) males, with ages: 20-50 years old - 6/41 (14,63%), 51-70 years old - 23/41 (56,09%) and over 71 years old - 12/41 (29,26%) patients. The histological types of CLD: B-cell non-Hodgkin's lymphoma - in 28/41 (68,29%) patients, T-cell non-Hodgkin's lymphoma - 2/41 (4,87%) patients, Hodgkin's lymphoma - 2/41 (4,87%), chronic lymphocytic leukemia - 7/41 (17,07%), Waldenström disease - 2/41 (4,87%) patients. Regarding the type of CLD, 19/41 (46,34%) of the patients have an aggressive type of CLD and 22/41 (53,65%) a non-aggressive type of CLD. The hepatitis viral infection distribution in our patients: 14/41 (34,14%) have HBV infection, 24/41 (58,53%) have HCV infection, double/triple association of viral infection was found in 3/41 (7,31%) patients. Within HBV infection subgroup 9/14 (64,28%) patients have an aggressive type of CLD and 5/14 (35,71%) patients have a non-aggressive type, whereas within the group with HCV infection we found a different distribution: 9/24 (37,5%) patients with aggressive type and 15/24 (62,5%) with non-aggressive type of CLD. The clinical parameters monitored were: B signs were present in 19/41 (43,34%) patients, the superficial or profound adenopathies -were found in 29/41 (70,73%) patients, hepatomegaly - in 38/41 (92,68%) patients, splenomegaly - in 21/41 (51,21%) patients, extra-nodal involvements in 10/41 (24,39%) patients and most frequent in the non-aggressive type of CLD - 6/10 (60%) patients. The hematological and biochemical parameters were: the presence of anemia and thrombocytopenia - found in a small number of patients; lymphocytosis - positive in 33/41 (80,48%) patients, most with HCV infection and non-aggressive type of disease, the presence of autoimmune hemolytic anemia - in 4/41 (9,75%) patients, cryoglobulins - 8/41 (19,51%) patients, all with HCV infection; also the liver function was monitored. Antiviral therapy was administered to 12/41 (29,26%) patients - Lamivudine to 8/41 (19,51%) patients and Ribavirine/Interferon to 4/41 (9,75%) patients. Chemotherapy was given in 32/41 (78%) patients. Monoclonal antibodies anti CD20 (Rituximab) therapy was associated in 6/41 (14,63%) patients. Conclusions. A high incidence in female sex of over 50 years old was noticed. A strong association between B-cell chronic lymphoproliferative disorders and hepatitis viral infection B, C, D was revealed, the most frequent being the C hepatitis virus, associated with the non-aggressive type of CLD, extra-nodal involvement, splenomegaly, lymphocytosis, cryoglobulins, cytolysis and colestasis. The clinical and biological disease history will be monitored during chemotherapy and antiviral treatment. [ABSTRACT FROM AUTHOR]