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Background: Autoimmune cytopenias (AIC) post hematopoietic stem cell transplant (HSCT) are rare but exceptionally challenging complications. Here, we report anti E, warm autoimmune hemolytic anemia (WAIHA), cold autoantibody, polyagglutination and immune thrombocytopenia in a 5-year-old boy with a history of chronic granulomatous disease, who underwent an ABO incompatible, mismatched (9/10) unrelated HSCT. Study design: Case report. Four months after HSCT, the patient was admitted for fever, fatigue, and decreased appetite. His original blood type was O+ and donor’s blood type B+. Prior to the admission he was transfused with 2 doses of red cells and 2 doses of platelets. On admission, hematopoietic chimera evaluation demonstrated 100% donor-derived cells. Initial laboratory work-up revealed white blood count 10.07x103/uL, hemoglobin 4.2 gm/dL, platelets 137,000 x103/uL, absolute retic count 0.123 x 10(6)/mcL, total bilirubin 1.8 mg/dl (mainly indirect) and lactate dehydrogenase 1,037 unit/L. Results: Patient’s red cells and saline suspended red cells spontaneously agglutinated in all blood bank tests precluding valid ABO/Rh type and direct antiglobulin test (DAT). An eluate, prepared from the patient’s red cells washed with saline, was reactive at 22C and following 37C incubation. Antibody testing on the patient’s plasma showed the presence of anti-E antibody. Titration and thermal amplitude studies demonstrated titer <2 and no reactivity at 30C and 37C, respectively. Donath Landsteiner test was negative. DTT treatment of the eluate showed significantly weakened reactivity at 22C and weak reactivity at PEG IAT. His red blood cells demonstrated strong reaction with AB (9/10) and B plasma (4/4). Lectin studies showed red cells reacting strongly with Arachis hypogea but were non-reactive with Glycine soja, Salvia sclarea, Salvia horminum. Several months later, the patient developed thrombocytopenia with platelet count of 45,000x103/uL. HLA antibody testing was negative, but SPRCA and ELISA tests were positive revealing antibodies against GPIIb/IIIa platelet antigens. Conclusion: This is a report of a 5-year-old male post-HSCT who developed overt hemolytic anemia with laboratory testing that demonstrated evidence of WAIHA, anti-E antibody, clinically insignificant cold autoantibody and polyagglutination (T activation/polyagglutination ruled out). Several months later, hemolysis subsided but immune mediated thrombocytopenia ensued. This case demonstrates the complexity of AIC that rarely develop in patients who underwent HSCT.
Hematology | Pathology
Cochran, Thomas; Oroszi, Gabor; Terwilliger, Nancy; Metzler, Gabe; and Music Aplenc, Lejla, "Case Report: Autoimmune anemia and thrombocytopenia following hematopoietic stem cell transplant" (2022). Posters. 306.