Miyata Toshiko, Masuzawa Mikio, Katsuoka Kensei, Higashihara Masaaki
Department of Dermatology, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan.
J Dermatol. 2008 Jul;35(7):456-61. doi: 10.1111/j.1346-8138.2008.00502.x.
Idiopathic myelofibrosis (IM) is a chronic myeloproliferative disorder and some cases of IM have extramedullary hematopoiesis. Extramedullary hematopoiesis is commonly seen in the liver, spleen and lymph nodes, but cutaneous extramedullary hematopoiesis (CEH) is very rare in cases of IM. We report a case of CEH in a 65-year-old Japanese woman with IM. This patient had many hard brownish nodules on her chest, abdomen and scalp. Histopathological examination of the nodule on her chest showed the existence of various stages of immature erythrocytes, leukocytes and megakaryocytes indicating that these hard nodules showed extramedullary hematopoiesis in the dermis. The proliferation of these immature cells in the dermis plays an important role in the pathogenesis of CEH. CEH is a very rare manifestation of IM and progresses slowly.
原发性骨髓纤维化(IM)是一种慢性骨髓增殖性疾病,部分IM病例存在髓外造血。髓外造血常见于肝脏、脾脏和淋巴结,但在IM病例中皮肤髓外造血(CEH)非常罕见。我们报告一例65岁日本女性IM患者发生CEH的病例。该患者胸部、腹部和头皮有许多坚硬的褐色结节。对其胸部结节进行组织病理学检查显示存在不同阶段的未成熟红细胞、白细胞和巨核细胞,表明这些坚硬结节在真皮层呈现髓外造血。这些未成熟细胞在真皮层的增殖在CEH的发病机制中起重要作用。CEH是IM一种非常罕见的表现形式,进展缓慢。