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Bone marrow necrosis in antiphospholipid syndrome
S Paydas1, R Koçak, S Zorludemir
1Department of Oncology, Cukurova University Faculty of Medicine, Balcali, Adana, Turkey.
Abstract:
Bone marrow necrosis (BMN) is a relatively rare entity and has been associated with a poor prognosis. It is most commonly found in patients with neoplastic disorders, severe infections and sickle cell anemia. An unusual case of antiphospholipid syndrome (APS) with extensive bone marrow necrosis is described in a 27 year old woman. The patient presented with severe pancytopenia, some cognitive impairment resulting from a previous cerebrovascular accident, fever, hypertension, dyspnoea, tachycardia, hepatosplenomegaly, and vaginal bleeding. Her laboratory findings included a strongly positive Coombs' test (anti-IgG and anti-C3d), a prothrombin time of 23 seconds and an activated partial thromboplastin time of 45 seconds. Anticardiolipin antibody tests were positive. Antinuclear and anti-DNA antibodies were negative but the anti-SM test was positive. A bone marrow biopsy specimen was reported as showing extensive necrosis. The patient was treated with steroids, transfusion, and plasma exchange with some clinical improvement but her pancytopenia did not respond and necessitated frequent transfusions. This case lends further support to the association between APS and BMN.
Insights
Antiphospholipid syndrome (APS) can cause extensive bone marrow necrosis (BMN), a rare condition linked to poor outcomes. This case highlights APS as a potential, though unusual, cause of BMN.
Area of Science:
- Hematology
- Rheumatology
- Pathology
Background:
- Bone marrow necrosis (BMN) is a rare condition often associated with neoplastic disorders, infections, or sickle cell anemia.
- Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent thrombotic events and/or pregnancy-related complications.
Observation:
- A 27-year-old woman with a history of cerebrovascular accident presented with severe pancytopenia, fever, hypertension, hepatosplenomegaly, and vaginal bleeding.
- Laboratory findings revealed a positive Coombs' test, prolonged coagulation times, positive anticardiolipin antibodies, and a positive anti-SM antibody test.
- Bone marrow biopsy confirmed extensive necrosis, despite negative antinuclear and anti-DNA antibodies.
Findings:
- The patient was diagnosed with antiphospholipid syndrome (APS) and extensive bone marrow necrosis (BMN).
- Treatment with steroids, transfusions, and plasma exchange provided partial clinical improvement but did not resolve the pancytopenia.
- This case underscores the association between APS and BMN, expanding the known spectrum of APS complications.
Implications:
- This case suggests that antiphospholipid syndrome should be considered in the differential diagnosis of unexplained bone marrow necrosis.
- Further research is warranted to elucidate the mechanisms linking APS and BMN and to optimize treatment strategies.
- Recognizing this association can lead to earlier diagnosis and potentially improved management for patients presenting with BMN.