Immune-related Pancytopenia: A Rare Case Report in Surabaya, Indonesia

Immune related pancytopenia (IRP) is a peripheral pancytopenia caused by bone marrow failure due to an autoimmune process. A 41 year old female presented with gum bleeding 4 days prior to admission. She had anemic conjunctiva and no organomegaly. Laboratory results were as follows Hb: 7.9 g/dL, WBC: 3,200/ìL, PLT: 6,000/ìL, IPF: 4.7% and RET: 0.44%. Bone marrow aspiration was dominated by clusters of erythroblastic islands and activated macrophages. Confirmatory examination with bone marrow mononuclear cells coombs test (BMMNC Coombs Test) showed positive results. The patient received dexamethasone and trasnfusions with both packed red cells (PRC) and platelet concentrate (PC) apheresis. Mechanism of pancytopenia in patients is an autoimmune process caused by an autoantibody towards hematopoeitic cells in the bone marrow. B lymphocytes over produces this autoantibody. Ig G autoantibody also activates macrophages that will phagocyte hematopoeitic cells, whereas the Ig M autoantibody activates the complement system that causes the lysis of hematopoietic cells. The diagnosis of IRP in this patient was based on peripheral pancytopenia with an increase of Erythroblastic Island clusters and activated macrophages in the bone marrow. The BMMNC Coombs Test confirmed the diagnosis.