Autoimmune Haemolytic Anemia

We present a unique case of a patient with a long-standing history of indolent chronic lymphocytic leukaemia (CLL) who suddenly developed autoimmune haemolytic anaemia after starting immune checkpoint inhibitor therapy for bladder cancer. He had no clear indication to start CLL-directed treatment based on current clinical practice guidelines; however, targeted treatment of CLL with ibrutinib proved to be effective in treating the haemolytic anaemia.

A 42-year-old diabetic man presented to the hospital with severe sepsis and multiorgan dysfunction. A probable respiratory source of sepsis was suspected because of suggestive clinical and radiological findings. He was critically ill and was therefore admitted to intensive care for further management including ventilatory support and renal replacement therapy. He was also found to have marked anaemia requiring multiple blood transfusions with clinical and laboratory evidence pointing towards severe haemolysis...

A thymoma belongs to rather rare tumors, although it holds the first place among primary mediastinal tumors in adults. The biological characteristics of thymomas are considerably divergent. The tumors are usually asymptomatic at the begining, later due to a local spread, symptoms from the infiltration or compression of adjacent structures arise, they metastasize rarely. Thymomas are often associated with various autoimmune or endocrinologic diseases. The authors present case of the patient with less common paraneoplasia - autoimmune hemolytic anemia (AIHA)...

OBJECTIVE: To analyze whether immune-mediated diseases (IMDs) occurs in sarcoidosis more commonly than expected in the general population, and how concomitant IMDs influence the clinical presentation of the disease. METHODS: We searched for coexisting IMDs in patients included in the SARCOGEAS-cohort, a multicenter nationwide database of consecutive patients diagnosed according to the ATS/ESC/WASOG criteria. Comparisons were made considering the presence or absence of IMD clustering, and odds ratios (OR) and their 95% confidence intervals (CI) were calculated as the ratio of observed cases of every IMD in the sarcoidosis cohort to the observed cases in the general population...

Autoimmune Hemolytic Anemia (AIHA) occurs in 10% to 25% of Chronic Lymphocytic Leukemia (CLL) while Direct Antiglobulin Test (DAT) positivity seen in 35% of cases. The prevalence and prognostic significance of DAT positivity is not well documented especially in Indian population. The present study was undertaken to know prevalence and prognostic significance of DAT positivity in CLL in India by associating it with stage and CD 38 expression. The study included fifty-eight newly diagnosed and untreated cases of CLL staged according to Binet and Rai system...

Atypical uremic haemolytic syndrome is a variant of thrombotic micro-andiopathy characterized by non-autoimmune hemolytic anemia, thrombocytopenia and acute renal failure as a result of excessive activation of the complement. Up to 60% of patients have mutations in the genes that encode the complement system. A disensing factor is required for its manifestation, including gestation. It is an entity with a high morbidity, which can decrease drastically if an early diagnosis is made and appropriate treatment is initiated...

The coronavirus disease 2019 (COVID-19) pandemic has caused significant morbidity and mortality worldwide. While patients with COVID-19 most frequently present with pneumonia, respiratory failure and acute respiratory distress syndrome, increasing cases of immune-mediated disorders such as autoimmune thrombocytopenia, haemolytic anaemia and antiphospholipid syndrome have been reported. In this article we describe a rare case of cold agglutinin syndrome (CAS) in a patient with COVID-19. The patient was a 77-year-old man with a history of glucose-6-phosphate dehydrogenase (G6PD) deficiency who presented with COVID-19 infection and acute respiratory failure...

A 25-year-old man who received alemtuzumab as induction therapy for a multivisceral transplant experienced delayed onset of warm autoimmune haemolytic anaemia and neutropaenia. Serological testing and bone marrow biopsy excluded alternative causes. Haemolysis was refractory to standard therapies including corticosteroids, intravenous immunoglobulin and rituximab. The patient was successfully treated with bortezomib, a proteasome inhibitor, and has remained well as an outpatient without evidence of ongoing haemolysis...

Long-term safety and efficacy of eltrombopag in adults with persitent/chronic primary immune thrombocytopenia (ITP) evaluated in EXTEND study, showed a high response rate (80%) but, in the clinical safety study, it was observed that 6% of the patients presented venous and arterial thrombotic events. In addition, in the course of the disease, autoimmune hemolytic anemia (Evans syndrome, ES) may occur and could increase the risk of thrombosis. We report an interesting case of splenic rupture due to massive intrasplenic arterial thrombosis in the course of ES in a patient with chronic ITP treated with eltrombopag...

Autoimmune hemolytic anaemia (AIHA) has traditionally been classified based on the temperature sensitivity of the autoagglutinins as warm (WAIHA), cold (CAIHA) and mixed type. Autoagglutinin may be of IgG or IgM type. The present prospective study was conducted to evaluate the profile of clinical picture, severity of haemolysis, treatment response of steroid. This study on patients of adult primary AIHA was conducted by taking complete history followed by detail physical examination. Laboratory investigations were performed to establish haemolytic anaemia and to assess severity of haemolysis...

A case of a 45-year-old male presentation with viral pneumonia with anemia and thrombocytopenia and was admitted to COVID-19 ICU. The blood bank encountered a discrepancy in blood grouping and cross-match, which were subsequently resolved. The patient presented to the emergency room with fever and shortness of breath. He had tachycardia, fever, and had an oxygen saturation of 88% on room air. His SARS-CoV-2 RT-PCR test was positive. Total and unconjugated bilirubin was raised. Due to the anemia and falling haemoglobin, he was not started on any anticoagulation...

BACKGROUND: Hemotropic mycoplasmas, previously classified in the genus Eperythrozoon, have been reported as causing human infections in Brazil, China, Japan and Spain. METHODS: In 2017, we detected DNA from "Candidatus Mycoplasma haemohominis" in the blood of a Melanesian patient from New Caledonia presenting with febrile splenomegaly,weight loss, life-threatening autoimmune haemolytic anemia and hemophagocytosis. The full genome of the bacterium was sequenced from a blood isolate...

Paroxysmal cold hemoglobinuria is a rare form of autoimmune hemolytic anemia caused by the Donath-Landsteiner autoantibody. The condition is characterized by the presence of an IgG biphasic hemolysin with specificity to the P blood group antigen. The antibody biphasic action may be demonstrated in the Donath-Landsteiner test. While paroxysmal cold hemoglobinuria can be manifested at any age, it typically appears in children following a viral upper respiratory syndrome or immunization, though rarely. This report describes a 23-months old girl presented with 5 days history of fever, erythrocytopenia, leukocytosis and occurrence of dark urine...

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a non-Langerhan cell histiocytosis which primarily involves lymph nodes. Extranodal involvement in the form of cutaneous plaques can occur and can pose a diagnostic challenge because of pleomorphic presentation and histopathological mimics. Rarely, systemic autoimmune involvement may complicate the disease process. We present a 28-year-old woman with slowly evolving scaly erythematous cutaneous plaques and fluctuating lymphadenopathy, associated with autoimmune haemolytic anaemia...

BACKGROUND: In idiopathic autoimmune haemolytic anaemia (AIHA haemolytic antibodies are directed to every type of red cellsWestern blot studies have shown antibody positivity towards red cell anion channel complex which also includes band 4.2 a protein with similarities to tissue trans glutaminase. OBJECTIVE: Evaluation of AIHA for anti tissue transglutaminase antibody (Anti tTG). MATERIALS & METHODS: Twenty three AIHA patients were tested along with routine hamatogical work up, for a series of auto antibodies and red cell eluates and serum from the patents were tested against solubilised group O red cell ghosts on western blot...

Retinopathy in the context of systemic lupus erythematosus (SLE) is associated with severe disease and poorer prognosis. We studied retinopathy in our cohort of Indian lupus patients. Four hundred and thirty-seven patients fulfilling the Systemic Lupus International Collaborating Clinics-American College of Rheumatology-2012 criteria, attending the department of Clinical Immunology were enrolled under this cross-sectional study. A comprehensive clinical (including ophthalmological) examination and immunological profile were performed...

Varicella is a common infectious exanthematous disease of children. Infection manifests as polymorphic maculopapulovesicular along with low grade fever, malaise and headache. Adults are less likely to be infected with varicella infection but once infected suffer disproportionately from serious complications like pneumonia, encephalitis, hepatitis and thrombocytopenia. Varicella hepatitis is generally a self-limiting disease with only a temporary subclinical rise in hepatic enzymes and are usually encountered however autoimmune hepatitis (AIH) which is an autoimmune disorder of unknown etiology affecting the liver is a very rare association of Varicella infection ...

BACKGROUND: Melanoma is one of the fastest growing neoplasms worldwide. Treatment of metastatic disease has swiftly shifted in the last decade from generally ineffective chemotherapy regimens to highly effective targeted treatments or immunotherapy, with a range of side effects that differ completely from those of previous treatments for this disease. CASE: We present a case of a 71-year-old man with diagnosis metastatic melanoma. This patient was treated with anti-CTLA-4 antibody ipilimumab...

Hematopoietic stem cell transplantation is usually performed without considering the ABO compatibility between donor and recipient. There are few studies analyzing ABO matching impact on transfusion outcome of umbilical cord blood transplantation (UCBT) recipients. The aim of this study was to analyze factors influencing transfusion outcome, highlighting the ABO matching between donor and recipient. This study has reviewed data from 318 patients who underwent single unit UCBT at la Fe University Hospital from January 2000 to December 2014...

INTRODUCTION: Haematological alterations such as anaemia, neutropenia and thrombocytopenia are frequent in Systemic Lupus Erythematosus (SLE). Ferritin being an acute phase reactant can be falsely elevated in lupus cases. AIM: To evaluate the haematological alterations and to re-categorise the types of anemia by soluble transferrin receptor levels in diagnosed cases of SLE. MATERIALS AND METHODS: A sample of 30 newly diagnosed ANA positive SLE patients was taken...