Primary Evans syndrome in an adult man

Evans syndrome (ES) is a simultaneous or subsequent development of two haematological disorders, autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP). It can be primary (idiopathic) or secondary (associated with an underlying disease). Primary Evans is a diagnosis of exclusion and has a poorer prognosis than AIHA or ITP alone. We present a 55-year-old man who presented with weakness and lethargy and was diagnosed to be suffering from primary ES.

Nivolumab-Induced Autoimmune Haemolytic Anaemia and Safety of Subsequent Use of Ipilimumab: A Case Report

Autoimmune haemolytic anaemia (AIHA) is a rare immune-related adverse event and appears to be more common with anti-PD1/PDL1 than anti-CTLA4. Little is known about the safety of re-treating with anti-PD1/PDL1 or changing to anti-CTLA4. We present a case of grade 4 AIHA due to nivolumab (PD1-inhibitor) treatment in a patient with melanoma for adjuvant setting after surgery and the safeness of subsequent treatment with ipilimumab (anti-CTLA4). After the remission of AIHA with steroids, ipilimumab was started with the rationale of its different mechanism of action. Fortunately, AIHA did not recur. The mechanism by which checkpoint inhibitors cause AIHA is likely by augmenting or redirecting immune surveillance, especially by activating pre-existing red blood cell autoantibodies, but further studies must be done. To our knowledge, this is the first case published in the literature with the change of immunotherapy treatment to anti-CTLA4.

How to use Donath-Landsteiner test to diagnose paroxysmal cold haemoglobinuria (PCH)

Paroxysmal cold haemoglobinuria (PCH) accounts for around a third of cases of autoimmune haemolytic anaemia in children. PCH is caused by an autoantibody that fixes complement to red cells at low temperatures and dissociates at warmer temperatures (a biphasic haemolysin), triggering complement-mediated intravascular haemolysis. Named the Donath-Landsteiner (D-L) antibody after its discoverers, it is usually formed in response to infection and demonstrates specificity for the ubiquitous red cell P-antigen. A D-L test can be used to detect the presence of the D-L autoantibody in the patients’ serum. Here we discuss the use of the D-L test in identifying PCH in a 2-year-old boy who presented with haemolytic anaemia. A summary of the key information can be found in the infographic.

An elderly man presented with autoimmune haemolytic anaemia- a consequence of severe corona virus disease 19 (COVID-19)

Autoimmune hemolytic anaemia (AIHA) can be caused by many diseases like connective tissue disease, lymphoproliferative disorder, certain infections and various medications. The coronavirus disease 19(COVID-19) can cause an increased risk of thrombosis. But, the association of AIHA with COVID-19 is not well understood. Here, in this case report a 45-year-old man who presented with fever, cough, anaemia and splenomegaly. On further investigation, he was confirmed as severe COVID-19 case with AIHA. Subsequently he was managed with prednisolone with good results. BSMMU J 2021; 14 (COVID -19 Supplement): 57-59