title: A case of bone marrow necrosis of an idiopathic aetiology: the report of a rare entity with review of the literature.
abstract: Bone Marrow Necrosis (BMN) is regarded as a rare entity in the aspirates and the trephine biopsies which are obtained from living patients and it is a rare antemortem diagnosis. Usually, BMN is associated with a poor prognosis and it has a 90% association with malignancies (mainly haematological). Its other causes include infections, drugs and other non malignant haematological causes. BMN which occurs due to unknown causes (idiopathic), is very rare. The present case report describes a case of BMN in a 14 year old male child from rural background, who came to the medicine outpatient department of a tertiary care centre in north India, with the complaints of generalized weakness, severe bone pains, especially in the ribcage area and fatigue. His peripheral blood film revealed pancytopaenia. His bone marrow aspiration yielded a watery, sero-sanguinous fluid from 2 different sites. Microscopy revealed "ghost like" haematopoietic cells lying in eosinophilic, proteinaceous material. Bone marrow biopsies from both the sites revealed extensive foci of gelatinous transformation and necrosis. A diagnosis of BMN was rendered. No underlying neoplastic / non neoplastic cause was identified, even after a thorough systematic investigation (haematological/biochemical/ radiological and immunological). Thus, a final diagnosis of BMN due to an idiopathic cause, was finally given. The patient was given supportive treatment, after which he was referred to a higher centre. BMN which arises idiopathically is usually very rare and often precedes the aetiological diagnosis and obscures the diagnosis. Traditionally, the prognoses of the patients with BMN were considered as poor, but with better treatment aids (chemotherapy and supportive treatment), the prognoses of these patients have been found to improve. Vigorous supportive care, along with a disease specific treatment, is the key to its management. This case was worth reporting, as no cause of BMN was elicited in it and we have summarized the signs and symptoms, the associations, haematological and other lab investigations and the role of radiology, which helped in its diagnosis.
authors: Associate Professor, Department of Pathology, Sri Guru Ram Das Institute of Medical Sciences and Research , Amritsar, Punjab, India .
authors: Tejinder Singh
authors: TS
authors: Bhasin
authors: Sonam
authors: S
authors: Sharma
authors: Manish
authors: M
authors: Chandey
authors: Puneet Kaur
authors: PK
authors: Bhatia
authors: Rahul
authors: R
authors: Mannan
keywords: Bone marrow; Idiopathic; Necrosis
references: Bone Marrow Transplant. 1997 May;19(10):1037-9
references: 9169650
references: Cancer. 2000 Apr 15;88(8):1769-80
references: 10760751
references: Am J Hematol. 1988 Dec;29(4):215-21
references: 3189318
references: Am J Hematol. 1992 May;40(1):42-6
references: 1566746
references: Am J Hematol. 1998 Sep;59(1):74-8
references: 9723581
references: Am J Hematol. 1994 Nov;47(3):225-8
references: 7942788
references: Med Pediatr Oncol. 1992;20(3):224-6
references: 1574033
references: Acta Haematol. 1980;64(4):232-5
references: 6781206
references: Am J Pathol. 1941 Jan;17(1):47-54.5
references: 19970543
references: Leuk Lymphoma. 1997 Jul;26(3-4):409-12
references: 9322906
references: Ann Saudi Med. 2000 Jan;20(1):78-82
references: 17322755
references: Ann Saudi Med. 1997 May;17(3):374-6
references: 17369747
references: Am J Forensic Med Pathol. 1990 Mar;11(1):83-8
references: 2305755
references: Cancer. 1983 Dec 1;52(11):2162-4
references: 6578869
references: Am J Med. 1976 Mar;60(3):361-8
references: 1062933