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Evans syndrome is defined as the concomitant or sequential association of autoimmune hemolytic anemia with immune thrombocytopenia, and less frequently autoimmune neutropenia. Here, we encountered a female patient who developed Evans syndrome associated with systemic lupus erythematosus (SLE) during follow-up for minimal change nephrotic syndrome (MCNS). The patient was diagnosed with MCNS at 14 years old. At 17 years old, she came to our hospital with fever, headache, and conjunctival icterus. Blood tests revealed hemolytic anemia, thrombocytopenia, and acute kidney injury. The direct Coombs test was positive, meeting six criteria for the diagnosis of SLE by the American College of Rheumatology. The patient was diagnosed with Evans syndrome secondary to SLE. The renal dysfunction and hematological abnormalities rapidly improved with the administration of prednisolone. Renal biopsy showed more than half of the glomeruli with endocapillary proliferation and wire-loop lesions. Some glomeruli showed segmental sclerosis. Immunofluorescence disclosed granular deposits of IgG, IgM, IgA, C3, and C1q in the glomerular basement membrane and mesangial region. Electron microscopy revealed dense electron deposits in the glomerular vascular endothelium. The patient was further diagnosed with Class IV-S (A/C)-type lupus nephritis and was treated with tacrolimus, with no relapse. Autoimmune disorders of the patient may be involved in the development of nephrotic syndrome. An interesting aspect of this case was the common pathogenesis of SLE and Evans syndrome, which are autoimmune diseases. In addition to the immunological disorders of the host, the influence of external factors may have contributed to the pathogenesis.

Secondary cold agglutinin syndrome (CAS) is autoimmune hemolytic anemia secondary to infections and lymphoid disorder. We here report the first Asian case of CAS secondary to novel coronavirus disease 2019 (COVID-19). A 72-year-old Japanese woman presented with a 2-week history of dyspnea and cough, and laboratory data revealed severe hemolytic anemia with a hemoglobin level of 4.7 g/dL. She was diagnosed with COVID-19, CAS, and monoclonal gammopathy of undetermined significance (MGUS). The anemia responded to corticosteroids administered for COVID-19 and required maintenance therapy. Although corticosteroids are not a standard therapy for CAS, they might be effective for CAS secondary to COVID-19 complicated with MGUS., Internal Medicine, 61(11), pp. 1789-1793; 202

Background and Aim: The prevalence and clinical significance of extrahepatic autoimmune diseases (EHAIDs) have not been evaluated in a large cohort of primary biliary cholangitis (PBC). Methods: The medical records of 1554 patients with PBC from 20 international centers were retrospectively reviewed. Development of decompensated cirrhosis (ascites, variceal bleeding, and/or hepatic encephalopathy) and hepatocellular carcinoma were considered clinical endpoints. Results: A total of 35 different EHAIDs were diagnosed in 440 (28.3%) patients with PBC. Patients with EHAIDs were more often female (92.5%vs86.1%,P < 0.001) and seropositive for anti-mitochondrial antibodies (88%vs84%,P = 0.05) and antinuclear antibodies and/or smooth muscle antibodies (53.8%vs43.6%,P = 0.005). At presentation, patients with EHAIDs had significantly lower levels of alkaline phosphatase (1.76vs1.98 x upper limit of normal [ULN],P = 0.006), aspartate aminotransferase (1.29vs1.50 x ULN,P < 0.001), and total bilirubin (0.53vs0.58 x ULN,P = 0.002). Patients with EHAIDs and without EHAIDs had similar rates of GLOBE high-risk status (12.3%vs16.1%,P = 0.07) and Paris II response (71.4%vs69.4%,P = 0.59). Overall, event-free survival was not different in patients with and without EHAIDs (90.8%vs90.7%,P = 0.53, log rank). Coexistence of each autoimmune thyroid diseases (10.6%), Sjogren disease (8.3%), systemic sclerosis (2.9%), rheumatoid arthritis (2.7%), systemic lupus erythematosus (1.7%), celiac disease (1.7%), psoriasis (1.5%), and inflammatory bowel diseases (1.3%) did not influence the outcome. Conclusions: Our study confirms that EHAIDs are frequently diagnosed in patients with PBC. The presence of EHAIDs may influence the clinical phenotype of PBC at presentation but has no impact on PBC outcome.

Background and Aim: The prevalence and clinical significance of extrahepatic autoimmune diseases (EHAIDs) have not been evaluated in a large cohort of primary biliary cholangitis (PBC). Methods: The medical records of 1554 patients with PBC from 20 international centers were retrospectively reviewed. Development of decompensated cirrhosis (ascites, variceal bleeding, and/or hepatic encephalopathy) and hepatocellular carcinoma were considered clinical endpoints. Results: A total of 35 different EHAIDs were diagnosed in 440 (28.3%) patients with PBC. Patients with EHAIDs were more often female (92.5%vs86.1%,P < 0.001) and seropositive for anti-mitochondrial antibodies (88%vs84%,P = 0.05) and antinuclear antibodies and/or smooth muscle antibodies (53.8%vs43.6%,P = 0.005). At presentation, patients with EHAIDs had significantly lower levels of alkaline phosphatase (1.76vs1.98 x upper limit of normal [ULN],P = 0.006), aspartate aminotransferase (1.29vs1.50 x ULN,P < 0.001), and total bilirubin (0.53vs0.58 x ULN,P = 0.002). Patients with EHAIDs and without EHAIDs had similar rates of GLOBE high-risk status (12.3%vs16.1%,P = 0.07) and Paris II response (71.4%vs69.4%,P = 0.59). Overall, event-free survival was not different in patients with and without EHAIDs (90.8%vs90.7%,P = 0.53, log rank). Coexistence of each autoimmune thyroid diseases (10.6%), Sjogren disease (8.3%), systemic sclerosis (2.9%), rheumatoid arthritis (2.7%), systemic lupus erythematosus (1.7%), celiac disease (1.7%), psoriasis (1.5%), and inflammatory bowel diseases (1.3%) did not influence the outcome. Conclusions: Our study confirms that EHAIDs are frequently diagnosed in patients with PBC. The presence of EHAIDs may influence the clinical phenotype of PBC at presentation but has no impact on PBC outcome.

A 65-year-old woman with systemic sclerosis and systemic lupus erythematosus developed acute kidney injury (AKI), Coombs-positive autoimmune hemolytic anemia and autoimmune thrombocytopenia; therefore, she was diagnosed with Evans syndrome (ES). Intravascular hemolysis was suggested as the cause of AKI based on the presence of acute tubular injury and trace hemosiderin deposits on the renal biopsy. The renal function, hemolytic anemia and thrombocytopenia were restored by an increased dose of glucocorticoids, hemodialysis, and plasma exchange. Although ES with severe hemolytic anemia is very rare, it is important to detect possible renal dysfunction when encountering patients with severe hemolysis.

Drug-induced autoimmune hemolytic anemia (AIHA) is a hematological disorder in which an immune destruction of red blood cells by antibodies occurs. Frequent agents that produce this disorder are antibiotics and chemotherapeutic agents; cases associated with different vaccines have been reported, including COVID-19 vaccines. We report a AHAI probably induced by the COVID-19 Vaxzevria vaccine., La anemia hemolítica autoinmune (AHAI) inducida por fármacos es un trastorno hematológico en cual se produce una destrucción inmnunitaria de los glóbulos rojos por anticuerpos. Los agentes más asociados son antibióticos y algunos quimioterápicos, pero también se han comunicado casos asociados con diferentes vacunas, incluido tras las vacunas frente a la COVID 19. Presentamos un caso de AHAI cuyo probable desencadenante fue la vacuna COVID 19 Vaxzevria.

Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infects humans through the angiotensin converting enzyme-2 (ACE-2) receptor expressed on many cells, including lymphocytes. In Covid-19 patients IL-6 is overexpressed, and hyperactivated plasmacytoid lymphocytes are detected in peripheral blood film. We hypothesize that, due to the unpredictable interaction between the new virus and the B cell lineage of infected patients, a cascade of out of control events can ensue, capable of determining unexpected pathologic disorders involving such lineage. Here we report two cases of autoimmune hemolytic anemia (AIHA) and two cases of B-cell hematological malignancies developed or reactivated during acute SARS-CoV-2 infection. The temporal relationship of the events may suggest a potential causal relationship between SARS-CoV-2 infection and the hematopoietic disorders. We suggest that special attention should be paid to COVID-19 patients with underlining B cell lineage disorders.

Background and Aim: The prevalence and clinical significance of extrahepatic autoimmune diseases (EHAIDs) have not been evaluated in a large cohort of primary biliary cholangitis (PBC). Methods: The medical records of 1554 patients with PBC from 20 international centers were retrospectively reviewed. Development of decompensated cirrhosis (ascites, variceal bleeding, and/or hepatic encephalopathy) and hepatocellular carcinoma were considered clinical endpoints. Results: A total of 35 different EHAIDs were diagnosed in 440 (28.3%) patients with PBC. Patients with EHAIDs were more often female (92.5% vs 86.1%, P < 0.001) and seropositive for anti-mitochondrial antibodies (88% vs 84%, P = 0.05) and antinuclear antibodies and/or smooth muscle antibodies (53.8% vs 43.6%, P = 0.005). At presentation, patients with EHAIDs had significantly lower levels of alkaline phosphatase (1.76 vs 1.98 x upper limit of normal [ULN], P = 0.006), aspartate aminotransferase (1.29 vs 1.50 x ULN, P < 0.001), and total bilirubin (0.53 vs 0.58 x ULN, P = 0.002). Patients with EHAIDs and without EHAIDs had similar rates of GLOBE high-risk status (12.3% vs 16.1%, P = 0.07) and Paris II response (71.4% vs 69.4%, P = 0.59). Overall, event-free survival was not different in patients with and without EHAIDs (90.8% vs 90.7%, P = 0.53, log rank). Coexistence of each autoimmune thyroid diseases (10.6%), Sjogren disease (8.3%), systemic sclerosis (2.9%), rheumatoid arthritis (2.7%), systemic lupus erythematosus (1.7%), celiac disease (1.7%), psoriasis (1.5%), and inflammatory bowel diseases (1.3%) did not influence the outcome. Conclusions: Our study confirms that EHAIDs are frequently diagnosed in patients with PBC. The presence of EHAIDs may influence the clinical phenotype of PBC at presentation but has no impact on PBC outcome.

Introduction: Sepsis commonly brings patients to the emergency department (ED). Patient outcomes can vary widely. In some cases, rare complications of sepsis such as autoimmune hemolytic anemia can occur.Case Report: A 68-year-old female presented with sepsis secondary to infected nephrolithiasis. The patient had signs and symptoms consistent with hemolysis upon arrival to the ED. Her hemolysis progressively worsened over a two-day period leading to a diagnosis of warm autoimmune hemolytic anemia. She responded well to treatment; however, her condition began to worsen due to a new infection caused by perforated colonic diverticula. The patient ultimately expired from complications of her perforated colonic diverticula.Conclusion: It is crucial that emergency physicians understand the risk factors, symptoms, pathophysiology, and treatment of this rare complication of sepsis so that favorable patient outcomes can be achieved.

Introduction: Sepsis commonly brings patients to the emergency department (ED). Patient outcomes can vary widely. In some cases, rare complications of sepsis such as autoimmune hemolytic anemia can occur.Case Report: A 68-year-old female presented with sepsis secondary to infected nephrolithiasis. The patient had signs and symptoms consistent with hemolysis upon arrival to the ED. Her hemolysis progressively worsened over a two-day period leading to a diagnosis of warm autoimmune hemolytic anemia. She responded well to treatment; however, her condition began to worsen due to a new infection caused by perforated colonic diverticula. The patient ultimately expired from complications of her perforated colonic diverticula.Conclusion: It is crucial that emergency physicians understand the risk factors, symptoms, pathophysiology, and treatment of this rare complication of sepsis so that favorable patient outcomes can be achieved.

The significant morbidity and mortality associated with iron overload can be reduced by effective iron chelation. Magnetic resonance imaging (MRI) provides accurate and reproducible iron load assessment. The aim of this epidemiological study was to assess the prevalence and severity of cardiac and hepatic siderosis by MRI and to evaluate the impact of MRI on clinical management in patients with transfusion-dependent anemia and non-transfusion-dependent thalassemia (NTDT). We enrolled 243 patients with myelodysplastic syndromes (MDS), thalassemia major (TM), NTDT or other chronic anemia. Overall, 10% and 48% had cardiac and hepatic siderosis, respectively. Mean liver iron concentration (LIC) was above target range in all groups; mean myocardial T2* was normal. Hepatic siderosis was more prevalent than myocardial siderosis in patients with MDS, occurring in 54.4% and 4.4% of patients, respectively. As also observed in patients with NTDT or other anemia, hepatic siderosis was present in a large proportion of MDS patients who were chelation naive (57.7%), as well as in patients receiving iron chelation therapy (ICT) (52.4%), despite a lower transfusion load compared with TM. Correlation between LIC and serum ferritin was observed across diseases; however, not all patients requiring ICT could be identified with serum ferritin alone, as serum ferritin underestimated LIC in 4.4% and overestimated LIC in 7.5% of patients. Exploratory analyses showed serum ferritin thresholds for liver siderosis detected by MRI at approximately 300 ng/mL higher in MDS than in TM. Most patients reported low-medium adherence to ICT; MRI assessment led to change in ICT in 46% of evaluable patients, including 52% of MDS patients. Accurate organ iron monitoring by MRI facilitated appropriate initiation of chelation, dose optimization and clinical decision making.Trial registration: ClinicalTrials.gov: NCT01736540.Copyright © 2019 the Author(s). Published by Wolters Kluwer Health, Inc.

The significant morbidity and mortality associated with iron overload can be reduced by effective iron chelation. Magnetic resonance imaging (MRI) provides accurate and reproducible iron load assessment. The aim of this epidemiological study was to assess the prevalence and severity of cardiac and hepatic siderosis by MRI and to evaluate the impact of MRI on clinical management in patients with transfusion-dependent anemia and non-transfusion-dependent thalassemia (NTDT). We enrolled 243 patients with myelodysplastic syndromes (MDS), thalassemia major (TM), NTDT or other chronic anemia. Overall, 10% and 48% had cardiac and hepatic siderosis, respectively. Mean liver iron concentration (LIC) was above target range in all groups; mean myocardial T2* was normal. Hepatic siderosis was more prevalent than myocardial siderosis in patients with MDS, occurring in 54.4% and 4.4% of patients, respectively. As also observed in patients with NTDT or other anemia, hepatic siderosis was present in a large proportion of MDS patients who were chelation naive (57.7%), as well as in patients receiving iron chelation therapy (ICT) (52.4%), despite a lower transfusion load compared with TM. Correlation between LIC and serum ferritin was observed across diseases; however, not all patients requiring ICT could be identified with serum ferritin alone, as serum ferritin underestimated LIC in 4.4% and overestimated LIC in 7.5% of patients. Exploratory analyses showed serum ferritin thresholds for liver siderosis detected by MRI at approximately 300 ng/mL higher in MDS than in TM. Most patients reported low-medium adherence to ICT; MRI assessment led to change in ICT in 46% of evaluable patients, including 52% of MDS patients. Accurate organ iron monitoring by MRI facilitated appropriate initiation of chelation, dose optimization and clinical decision making.Trial registration: ClinicalTrials.gov: NCT01736540.Copyright © 2019 the Author(s). Published by Wolters Kluwer Health, Inc.

Background Fingolimod is an oral sphingosine-1-phosphate-receptor modulator which causes lymphocyte sequestration in lymph nodes. It was approved for relapsing Multiple Sclerosis (MS) following evidence it reduced relapse rates by 50%1,2. The Therapeutic Goods Administration (TGA) of Australia as of September 2015 was aware of only one case where fingolimod preceded ITP by five weeks. Aims To report three cases of ITP associated with fingolimod. Methods We retrospectively reviewed three cases of fingolimod associated ITP who presented to Monash Health between 2013 and 2015. Results Cases are described in Table 1. None were on any medications known to cause ITP and routine investigations were non-contributory. All cases were treated with immunosuppression. Case 1 successfully weaned prednisolone after fingolimod cessation whilst case 2 had a slower wean whilst continuing fingolimod therapy. Case 3 had more refractory ITP and re-exposure to fingolimod worsened thrombocytopenia. Possible mechanisms of the potential association between fingolimod and ITP remain unclear. One possible theory is immune dysregulation given fingolimod has been associated with autoimmune haemolytic anaemia3 and haemophagocytic syndrome4,5. Another could be that it merely highlights autoimmune clustering. (Table Presented) Summary and Conclusion In conclusion, our cases highlight that clinicians should be aware of the possible association between ITP and fingolimod although the mechanism for this remains unclear.

Background Fingolimod is an oral sphingosine-1-phosphate-receptor modulator which causes lymphocyte sequestration in lymph nodes. It was approved for relapsing Multiple Sclerosis (MS) following evidence it reduced relapse rates by 50%1,2. The Therapeutic Goods Administration (TGA) of Australia as of September 2015 was aware of only one case where fingolimod preceded ITP by five weeks. Aims To report three cases of ITP associated with fingolimod. Methods We retrospectively reviewed three cases of fingolimod associated ITP who presented to Monash Health between 2013 and 2015. Results Cases are described in Table 1. None were on any medications known to cause ITP and routine investigations were non-contributory. All cases were treated with immunosuppression. Case 1 successfully weaned prednisolone after fingolimod cessation whilst case 2 had a slower wean whilst continuing fingolimod therapy. Case 3 had more refractory ITP and re-exposure to fingolimod worsened thrombocytopenia. Possible mechanisms of the potential association between fingolimod and ITP remain unclear. One possible theory is immune dysregulation given fingolimod has been associated with autoimmune haemolytic anaemia3 and haemophagocytic syndrome4,5. Another could be that it merely highlights autoimmune clustering. (Table Presented) Summary and Conclusion In conclusion, our cases highlight that clinicians should be aware of the possible association between ITP and fingolimod although the mechanism for this remains unclear.

Hairy cell leukemia (LCV) and splenic marginal zone lymphoma (LME) are two lymphoproliferative disorders that usually have in common a similar clinical presentation with splenomegaly, cytopenias and an indolent clinical course. It is essential to make a proper differential diagnosis, since the therapeutic approach and prognosis differ between both entities. LCV responds to treatment with nucleoside analogs, while LME treatment options include splenectomy or rituximab. The diagnostic approach includes imaging studies, flow cytometry and pathology of bone marrow, although the results of these methods are not always conclusive. We report a clinical case that had an acute and severe presentation, with autoim-mune hemolytic anemia and it also implied a diagnostic challenge in which the use of a novel method was helpful to arrive to a definitive diagnosis and an accurate treatment, La leucemia de células vellosas (LCV) y el linfoma de la zona marginal esplénico (LME) son dos neoplasias linfoproliferativas que con frecuencia comparten una presentación clínica semejante, con esplenomegalia, citopenias y un curso clínico indolente. Es fundamental realizar un diagnóstico diferencial adecuado, ya que el enfoque terapéutico y el pronóstico difieren entre estas dos entidades. La LCV responde al tratamiento con análogos nucleosídicos, mientras que para el LME las opciones de tratamiento comprenden la esplenectomía o el uso de rituxi-mab monodroga. El abordaje diagnóstico incluye estudios de imágenes, citometría de flujo y anatomía patológica de médula ósea, aunque los resultados de estos métodos no siempre son concluyentes. Reportamos un caso clínico con una presentación aguda y severa (anemia hemolítica autoinmune) que resultó un reto diagnóstico en el cual el uso de un método novedoso fue de ayuda para arribar al diagnóstico definitivo y al tratamiento adecuado.

Embedded Image Introduction Certain patients with antibody-mediated autoimmune disease exhibit poor responses to conventional immunosuppression. Proteasome inhibitors (PI) have been prospectively evaluated in humoral graft rejection, where rapid alloantibody depletion occurs in the context of combined immunosuppression. In addition to killing antibody-producing cells, PIs deplete autoreactive T-cells, suppress inflammatory NFkB signaling and modulate MHC class I antigen presentation. The durability of rituximab-based B-cell depletion may be abrogated by promotion of long-lived autoreactive (CD20 neg) plasma cell development. Therefore there is a strong rational for utilising PIs in antibody mediated autoimmune disease. Based on our initial experience re-purposing bortezomib (BTZ) to deplete ADAMTS-13 antibodies in thrombotic thrombocytopenic purpura (TTP) (Shortt et al NEJM 2013; 69: 90-2.), we have utilized off-label BTZ in cases of severe refractory antibody-mediated hematological disease via a compassionate access program. We report the first case-series of BTZ use in this broader patient group. Patients & Methods Outcome data were collected for all patients treated between 2012 and 2015 with off-label BTZ salvage for autoantibody-mediated hematological disease across a combined health-care network in the Australian state of Victoria (catchment area >1.5 million patients; 3 major academic centers). Eligible patients were refractory to standard of care and at risk of severe morbidity/mortality from underlying disease or immunosuppression. All patients demonstrated on-going autoantibody production despite rituximab-based B-cell depletion. We sought to correlate biological responses (e.g. reductions in autoantibody titer) with clinical efficacy (e.g. remission rates and capacity to wean concurrent immunosuppression) and to document BTZ toxicities. Compassionate BTZ access was subject to institutional drug and therapeutic committee review and Australian Therapeutic G

Embedded Image Introduction Certain patients with antibody-mediated autoimmune disease exhibit poor responses to conventional immunosuppression. Proteasome inhibitors (PI) have been prospectively evaluated in humoral graft rejection, where rapid alloantibody depletion occurs in the context of combined immunosuppression. In addition to killing antibody-producing cells, PIs deplete autoreactive T-cells, suppress inflammatory NFkB signaling and modulate MHC class I antigen presentation. The durability of rituximab-based B-cell depletion may be abrogated by promotion of long-lived autoreactive (CD20 neg) plasma cell development. Therefore there is a strong rational for utilising PIs in antibody mediated autoimmune disease. Based on our initial experience re-purposing bortezomib (BTZ) to deplete ADAMTS-13 antibodies in thrombotic thrombocytopenic purpura (TTP) (Shortt et al NEJM 2013; 69: 90-2.), we have utilized off-label BTZ in cases of severe refractory antibody-mediated hematological disease via a compassionate access program. We report the first case-series of BTZ use in this broader patient group. Patients & Methods Outcome data were collected for all patients treated between 2012 and 2015 with off-label BTZ salvage for autoantibody-mediated hematological disease across a combined health-care network in the Australian state of Victoria (catchment area >1.5 million patients; 3 major academic centers). Eligible patients were refractory to standard of care and at risk of severe morbidity/mortality from underlying disease or immunosuppression. All patients demonstrated on-going autoantibody production despite rituximab-based B-cell depletion. We sought to correlate biological responses (e.g. reductions in autoantibody titer) with clinical efficacy (e.g. remission rates and capacity to wean concurrent immunosuppression) and to document BTZ toxicities. Compassionate BTZ access was subject to institutional drug and therapeutic committee review and Australian Therapeutic G

Introduction: Autoimmune hemolytic anemia characterized by production of antibodies that attach to Red cells resulting in their destruction causing anemia. AIHA could be primary or secondary to some other cause. The positive DCT is hallmark for diagnosis of AIHA. Aim of Study: To perform clinical and hematological study of AIHA and from available data to derive necessary conclusion for accurate diagnosis of AIHA. Material and Methods: The prospective study was carried out in blood bank at tertiary care hospital in positive DCT cases from suspected AIHA cases. The detail history was taken, clinical presentation studied and necessary laboratory investigations like peripheral smear examination, Reticulocyte count, Serum bilirubin, Serum LDH etc done and all data tabulated and conclusion was made for diagnosing AIHA accurately. DCT was performed both manually and using gel card technology. Conclusion: Females and age group between 20 to 30 have more predilections for developing AIHA. Secondary cases are more common and severity of presentation was aggressive in primary cases as compared to secondary. Such type of study helpful in diagnosis AIHA accurately and delay in treatment can be avoided and awareness can be created.

Introduction: Autoimmune hemolytic anemia characterized by production of antibodies that attach to Red cells resulting in their destruction causing anemia. AIHA could be primary or secondary to some other cause. The positive DCT is hallmark for diagnosis of AIHA. Aim of Study: To perform clinical and hematological study of AIHA and from available data to derive necessary conclusion for accurate diagnosis of AIHA. Material and Methods: The prospective study was carried out in blood bank at tertiary care hospital in positive DCT cases from suspected AIHA cases. The detail history was taken, clinical presentation studied and necessary laboratory investigations like peripheral smear examination, Reticulocyte count, Serum bilirubin, Serum LDH etc done and all data tabulated and conclusion was made for diagnosing AIHA accurately. DCT was performed both manually and using gel card technology. Conclusion: Females and age group between 20 to 30 have more predilections for developing AIHA. Secondary cases are more common and severity of presentation was aggressive in primary cases as compared to secondary. Such type of study helpful in diagnosis AIHA accurately and delay in treatment can be avoided and awareness can be created.

Intravascular large cell lymphoma (IVLCL) is a rare disease characterized by proliferation of malignant lymphocytes within the small blood vessel lumens. The association of IVLCL with autoimmune hemolytic anemia (AIHA) has been described in a single case report, but the true prevalence of this co-occurrence is not known because of declining autopsy rates. Here, we report a case of a 41-year-old woman who carried a diagnosis of AIHA for 2 years, with repeated hemolytic episodes that were initially well controlled with immunomodulatory treatment. At her last presentation, the patient developed rapidly progressive neurologic symptoms and leukoencephalopathy on MRI; she died 4 weeks later with a clinical impression of thrombotic microangiopathy, a known complication of AIHA. At autopsy, the brain showed widespread platelet thrombi and intraparenchymal hemorrhages characteristic of this disorder. In addition, there was evidence of a clinically unsuspected IVLCL, most likely of B-cell lineage. This case illustrates a potential association between IVLCL and AIHA, highlights the need for broad differential diagnosis in cases with atypical disease presentation or progression, and underlines the importance of autopsy in establishing the full cause of morbidity and mortality.

Hypereosinophilic syndrome (HES) encompasses numerous diverse conditions resulting in peripheral hypereosinophilia that cannot be explained by hypersensitivity, infection, or atopy and that is not associated with known systemic diseases with specific organ involvement. HES is often attributed to neoplastic or reactive causes, such as chronic eosinophilic leukemia, although a majority of cases remains unexplained and are considered idiopathic. Here, we review the current diagnosis and management of HES and present a unique case of profound hypereosinophilia associated with warm autoimmune hemolytic anemia requiring intensive management. This case clearly illustrates the limitations of current knowledge with respect to hypereosinophilia syndrome as well as the challenges associated with its classification and management.

Hypereosinophilic syndrome (HES) encompasses numerous diverse conditions resulting in peripheral hypereosinophilia that cannot be explained by hypersensitivity, infection, or atopy and that is not associated with known systemic diseases with specific organ involvement. HES is often attributed to neoplastic or reactive causes, such as chronic eosinophilic leukemia, although a majority of cases remains unexplained and are considered idiopathic. Here, we review the current diagnosis and management of HES and present a unique case of profound hypereosinophilia associated with warm autoimmune hemolytic anemia requiring intensive management. This case clearly illustrates the limitations of current knowledge with respect to hypereosinophilia syndrome as well as the challenges associated with its classification and management.

Intravascular large cell lymphoma (IVLCL) is a rare disease characterized by proliferation of malignant lymphocytes within the small blood vessel lumens. The association of IVLCL with autoimmune hemolytic anemia (AIHA) has been described in a single case report, but the true prevalence of this co-occurrence is not known because of declining autopsy rates. Here, we report a case of a 41-year-old woman who carried a diagnosis of AIHA for 2 years, with repeated hemolytic episodes that were initially well controlled with immunomodulatory treatment. At her last presentation, the patient developed rapidly progressive neurologic symptoms and leukoencephalopathy on MRI; she died 4 weeks later with a clinical impression of thrombotic microangiopathy, a known complication of AIHA. At autopsy, the brain showed widespread platelet thrombi and intraparenchymal hemorrhages characteristic of this disorder. In addition, there was evidence of a clinically unsuspected IVLCL, most likely of B-cell lineage. This case illustrates a potential association between IVLCL and AIHA, highlights the need for broad differential diagnosis in cases with atypical disease presentation or progression, and underlines the importance of autopsy in establishing the full cause of morbidity and mortality.

A 53-years-old man has a dysentery since two weeks. The blood test shows Coombs-positive hemolytic anemia and inflammation. Autoimmune hemolytic anemia (AIHA) is treated with corticosteroid. A colonoscopy reveals an ulcerative colitis. The evolution of the patient is complicated by a spontaneous digestive perforation treated by total proctocolectomy. After this intervention, there is a resolution of the AIHA and the patient is gradually weaned from corticosteroids. AIHA is a rare extra-intestinal manifestation of inflammatory bowel disease essentially ulcerative colitis. Identification of this cause of secondary AIHA is important for the therapeutic strategy. However treatment is nonspecific and based on low levels of evidence., SCOPUS: ar.j, info:eu-repo/semantics/published

Chronic paroxysmal intracranial hypertension leading to syncope is a phenomenon not reported previously in patients with refractory cerebral venous sinus thrombosis. We report a case of paroxysmal intracranial hypertension leading to syncopal episodes in a patient with idiopathic autoimmune hemolytic anemia and venous sinus thrombosis. This case demonstrates that intermittent elevations in intracranial pressure can lead to syncope in patients with venous sinus thrombosis and emphasizes the importance of considering this potentially treatable etiology of syncopal episodes.

A young female presented with acute abdominal pain of two days duration consistent with acute pancreatitis. During her stay in the hospital she had a sudden drop in hemoglobin to 6 g/dl without any overt blood loss. On evaluation, it was evident that she had acute pancreatitis, in addition to displaying features of autoimmune hemolytic anemia. She had been a known case of systemic lupus erythematosus (SLE) and had discontinued her treatment. She was managed with methylprednisolone pulse therapy. Her clinical condition improved, and she has been regularly attending our clinic for the last 2 years. According to a literature search in Medline, it would appear that this is the first report of a case in which SLE with autoimmune hemolytic anemia has been associated with acute pancreatitis in a single case., Eine junge Frau stellte sich mit akuten Bauchschmerzen von zwei Tagen Dauer, die auf eine akute Pankreatitis hinwiesen, vor. Während ihres Aufenthaltes im Krankenhaus erlitt sie einen plötzlichen Abfall des Hämoglobins auf 6 mg/dl ohne offenen Blutverlust. Die Untersuchung ergab, dass sie eine akute Pankreatitis hatte und zusätzlich Symptome einer autoimmunhämolytischen Anämie zeigte. Die Patientin hatte eine bekannte Vorgeschichte von systemischem Lupus erythematodes (SLE) mit Behandlungsabbruch. Sie wurde mit Methylprednisolon-Pulstherapie behandelt. Ihre klinischer Zustand verbesserte sich, und sie wird seit den letzten 2 Jahren regelmäßig in unserer Klinik untersucht. Nach einer in Medline durchgeführten Literaturrecherche scheint es, dass dies der erste Bericht über einen Fall ist, in dem SLE mit autoimmunhämolytischer Anämie und akuter Pankreatitis zusammen auftritt.

A case of systemic lupus erythematosus (SLE) with autoimmune hemolytic anemia (AIHA) and idiopathic thrombocytopenic purpura (ITP) is reported. The patient was a 21-year-old female, who had been diagnosed as having ITP at the age of 9, and SLE at the age of 11. Oral administration of predonisolone at ordinary dosage had been given for a decade. When she was 19 years old, she suffered from AIHA, and two years later, she suffered from severe thrombocytopenia. In both episodes, methylpredonisolone pulse therapy was carried out. The therapy was markedly effective for hemolysis and thrombocytopenia. This clinical combination of AIHA and ITP known as "Evans' syndrome" is associated with organ specific autoantibodies. Some considerations of Evans' syndrome and SLE are described.

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