Corella F, Barnadas M A, Bordes R, Curell R, Espinosa I, Vergara C, Alomar A
Servicio de Dermatología, Hospital de la Santa Creu i Sant Pau, Barcelona, España.
Actas Dermosifiliogr. 2008 May;99(4):297-300.
Cutaneous extramedullary hematopoiesis is a rare manifestation of chronic myeloproliferative processes, mainly chronic idiopathic myelofibrosis. In adults, it manifests as macules, papules, nodules, and ulcers on the trunk. The lesions usually appear soon after diagnosis and the possibility of a relationship between splenectomy and the appearance of extramedullary foci of hematopoiesis is still debated. Diagnosis is based on histopathology showing an infiltrate with different combinations of myeloid and erythroid cell precursors and megakaryocytes. Symptomatic treatment is provided alongside treatment of the underlying disease. We report a new case associated with chronic idiopathic myelofibrosis in which foci of cutaneous extramedullary hematopoiesis were observed 9 years after initial diagnosis. The lesions were progressive and the patient went on to develop acute myeloid leukemia.
皮肤髓外造血是慢性骨髓增殖性疾病的一种罕见表现,主要见于慢性特发性骨髓纤维化。在成人中,表现为躯干上的斑疹、丘疹、结节和溃疡。这些病变通常在诊断后不久出现,脾切除术与髓外造血灶出现之间的关系仍存在争议。诊断基于组织病理学检查,显示有髓系和红系细胞前体及巨核细胞的不同组合浸润。在治疗基础疾病的同时给予对症治疗。我们报告一例与慢性特发性骨髓纤维化相关的新病例,在初次诊断9年后观察到皮肤髓外造血灶。病变呈进行性发展,患者继而发展为急性髓系白血病。