PMC5435183_01
Severe autoimmune hemolytic anemia associated with eosinophilic granulomatosis with polyangiitis. A, B. Peripheral blood smear is shown at low and high power magnification demonstrating eosinophilia, significant RBC agglutination at room temperature (A), and predominant spherocytes (B). Bone marrow core biopsy demonstrates hypercellularity (C) with eosinophilic and erythroid hyperplasia (D). E, F. Left thigh skin biopsy demonstrates eosinophilic infiltration in a small vessel distribution*.
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F1
A peripheral blood smear demonstrated full field spherocytes and significant agglutination at room temperature (figure 1A,B). A skin biopsy of the plaque-like rash on the patient's left thigh was performed demonstrating small vessel vasculitis predominantly composed of eosinophils (figure 1E,F).
Warm IgM AIHA responsive to eculizumab and rituximab. Hemoglobin is shown during the patient's hospital and outpatient course. Treatments are indicated with doses included below.. Cyclophosphamide: 700mg/m2, Eculizumab: 600mg IV, methylprednisolone: 40mg IV, methylprednisolone (high) (1000mg IV), PRBC: 3 units, prednisone 1mg/kg, rituximab: 375mg/m2. PLEX: plasma exchange; PRBC: packed red blood cells; RTX: rituximab
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F2
Despite this treatment and the transfusions, the patient's hemoglobin decreased to 5-6 g/dL (figure 2). However, with daily plasma exchange and another round of transfusions, the patient's hemoglobin only transiently increased from roughly 6 g/dL to 8 g/dL with the increase only lasting several hours (figure 2).