Extensive bone marrow necrosis with sepsis in primary thrombocytemia: a case report and literature review

La Revue de médecine interne
E RivièreA Boyer

Abstract

Bone marrow necrosis is a very rare condition which is characterized by a necrosis of hematopoietic progenitors, adipocytes and reticulin network. We report a 62-year-old woman admitted to an intensive care unit for an essential thrombocytemia associated with bone marrow necrosis complicated by septic shock and progressive multi-organ failure. To our knowledge, this is the second case reported in the literature. The clinical presentation of bone marrow necrosis includes non-specific symptoms such as fever, bone pain and sometimes a clinically significant medullar insufficiency syndrome. Biology can reveal cytopenias, elevated LDH and alkaline phosphatase serum levels. The diagnosis is confirmed by bone marrow trephine biopsy. Bone marrow necrosis is classified as extensive if more than 50% of the bone marrow biopsy show necrosis. Haematological malignancies (particularly leukaemia), and solid malignant tumours (particularly gastro-intestinal or lung cancers) represent up to 90% of aetiologies and must be actively researched. Also, sickle cell disease and catastrophic anti-phospholipid syndrome must also be investigated. Essential thrombocytemia remains an exceptional cause of bone marrow necrosis. Overall the prognosis of bone ...Continue Reading

References

Jan 18, 1979·European Journal of Pediatrics·H G Terheggen, F Lampert
Nov 28, 2000·The American Journal of the Medical Sciences·K I Ataga, E P Orringer
Mar 1, 2002·The New England Journal of Medicine·Michelle A ElliottAyalew Tefferi
Sep 5, 2002·American Journal of Hematology·Semra PaydasFiliz A Bolat
Dec 23, 2004·European Journal of Haematology·Alfredo N C SantanaRodrigo A Brandão-Neto
Jan 24, 2007·AJR. American Journal of Roentgenology·Yu Ming TangDevinder Gill
Dec 29, 2007·The Journal of Pathology·J R Bradley
Apr 18, 2008·Blood Reviews·Imad UthmanMunther Khamashta
Feb 8, 2011·La Revue de médecine interne·S JosseV Queyrel

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