Non-caseating granulomata associated with hypocellular myelodysplastic syndrome.
Non-caseating granuloma (NCG) remains a histopathological hallmark for sarcoidosis. Although the exact mechanism for NCG formation is unknown, the pathogenesis may involve a disordered antigen presentation in the monocyte/macrophage system, functional abnormalities in activated T-lymphocytes and uncontrolled cytokine production. Similar immunological dysfunction has been described in myelodysplastic syndrome (MDS). However, the association of NCG and MDS is rarely documented. We report a case of hypocellular MDS associated with generalized NCG. Despite treatment for both sarcoidosis and tuberculosis, the patient failed to respond. A clonal myeloid disorder which was initially suppressed by T-cell immunosurveillance evolved after treatment with anti-thymocyte globulin. Although the coexistence of sarcoidosis remains a possibility, the lack of supportive clinical evidence of sarcoidosis, the abnormal appearances of the bone marrow, together with the failure to improve on high-dose steroid favour the clonal myeloid disorder as the sole pathology.
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Department of Haematological Medicine, King's College Hospital, Denmark Hill, London SE5 9RS. tsfng@hotmail.com
Yeghen
T
T
Pagliuca
A
A
Gillett
D S
DS
Mufti
G J
GJ
D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006099:Granuloma / Q000209:etiology*; D006801:Humans; D008297:Male; D009190:Myelodysplastic Syndromes / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D012507:Sarcoidosis / Q000175:diagnosis