Ruberto E, Espinola R, Brusco J, Bacchiocchi M, Zoppi J, Paz R
Servicio de Hematología, Hospital Privado de Comunidad, Córdoba, Argentina.
Sangre (Barc). 1995 Apr;40(2):157-60.
Idiopathic myelofibrosis (IMF) is a clonal chronic myeloproliferative syndrome characterized by the proliferation of the three haemopoietic series and the marrow connective tissue and by the development of extramedullary haemopoiesis in the liver, spleen and lymph nodes. Cutaneous extramedullary haemopoiesis is an uncommon event and we could not find any reported cases of testicular involvement in this disease. We report the case of a 28 year-old male with diagnosis of idiopathic myelofibrosis in November 1988. During the course of the disease, three years later, he developed a tumor on his right testis. Histologic examination showed extramedullary haemopoiesis with cells of the myeloid, erythroid and megakaryocyte series, in the interstice. Eight months later, numerous red-purple papules and nodules developed on the patients's trunk. The biopsy of a skin lesion revealed an infiltration of the dermis by myeloid, erythroid cells and few megakaryocytes. The patient's clinical condition worsened, and he died in February 1993 following progressive deterioration of the general condition. We describe a case of IFM with extramedullary hemopoiesis involving the skin and the testis pointing out the rarity of these localization.
原发性骨髓纤维化（IMF）是一种克隆性慢性骨髓增殖性综合征，其特征为三个造血系列及骨髓结缔组织的增殖，以及肝脏、脾脏和淋巴结的髓外造血。皮肤髓外造血是一种罕见情况，我们未发现有关于该疾病累及睾丸的报道病例。我们报告一例1988年11月诊断为原发性骨髓纤维化的28岁男性病例。在疾病过程中，三年后他右侧睾丸出现一个肿瘤。组织学检查显示间质中有髓系、红系和巨核细胞系细胞的髓外造血。八个月后，患者躯干出现大量红紫色丘疹和结节。皮肤病变活检显示真皮有髓系、红系细胞及少量巨核细胞浸润。患者临床状况恶化，1993年2月因全身状况进行性恶化而死亡。我们描述一例原发性骨髓纤维化伴皮肤和睾丸髓外造血的病例，指出这些部位受累的罕见性。
