Hematoidin Pigment with Bone Marrow Necrosis in Sickle Cell Anemia

Excerpt

A 36-year-old man, case of sickle cell disease presented with abdominal distension, jaundice, pain in bilateral thighs and lower back ache for last 4–5 days. Within 2 days of admission there was fall in hemoglobin (Hb-9.3 g/dl, to Hb-3.8 g/dl); rise in total bilirubin (8.1–21.3 mg/dl) and LDH (1771–4588 IU/l) levels. Peripheral blood demonstrated normocytic normochromic red cells along with few sickle cells, polychromatophils and Howell Jolly bodies; leucopenia with lymphopenia and reduced platelet count. HPLC revealed increased HbS (79.4%) and HbF (12.40%). A diagnosis of vaso-occlusive crisis with hyperhemolysis was made and RBC exchange transfusion done following which HbS levels decreased (~ 21.4%) and HbA levels increased. The patient underwent bone marrow for cytopenias. Bone marrow aspiration revealed normoblastic erythroid hyperplasia with normal myeloid maturation and megakaryocytes. Bone marrow biopsy demonstrated marrow necrosis (Fig. 1a) and within its milieu thread like, golden brown, crystalline, filaments of hematoidin were seen. This pigment also elicited giant cell reaction (Fig. 1b). The color of the pigment was maintained through multiple histochemical stains, including hematoxylin and eosin (H&E), Prussian blue (Fig. 1c) and bile stain.

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