Severe bone marrow necrosis without suggestive features

Abstract

Bone marrow necrosis (BMN) is a rare disorder defined pathologically as necrosis of myeloid tissue and medullary stroma in large areas of the bone marrow with preservation of bone [1]. Although postmortem reports show a higher prevalence of this disorder, the prevalence of BMN is highly variable and a relatively rare entity diagnosed during life [2]. A 19-year-old boy was hospitalized in June 2004 with a 1-week history of fever, anorexia, dysphagia, and cough. He was diagnosed as having HL 3 years ago. Because optimal response was not achieved with chemotherapies, autologous bone marrow transplantation was performed in September 2003. Physical examination at the time of presentation revealed fever 38.78C, pale appearance, extensive cutanaous ecchymoses, left inguinal lymphadenopathy, and hepatomegaly. Laboratory findings were as follows: hemoglobin 4.6 g/dl; white blood cell count 0.13 109 /L; platelet count 9 109 /L; lactate dehydrogenase (LDH) 144 UI/L (125–250), and alkaline phosphatase (ALP) 74 UI/L (35–150). ESR was 110 mm/h. Thorax and abdominal CT scans revealed progression of disease. The patient had febrile neutropenia and was put on antibiotherapy. All cultures for bacteria and fungi were negative as well as serologic tests for viruses including those that can cause cytopenia. Bone marrow aspiration resulted in ‘‘dry tap’’ and a biopsy specimen from the posterior iliac crest showed the necrosis of bone marrow and trabeculae. In the biopsy specimen, the marrow space was totally replaced by coagulative necrotic cells with preservation of only the basic outlines. Bone marrow scintigraphy showed low uptakes within the bone marrow, especially in the iliac bone, with increased uptakes in the axial bone and hand and foot phalanx. Two weeks later, the patient succumbed with massive pulmonary hemorrhage albeit supportive care.