以全身紫红色结节为首发表现的慢性粒单核细胞白血病1例及文献复习

Chronic myelomonocytic leukemia presenting with diffuse purple-red nodules as initial clinical manifestation:a case report and literature reviews

报告1例以全身紫红色结节为首发表现的慢性粒单核细胞白血病。患者女,75岁。因全身紫红色结节伴瘙痒10 d就诊。皮肤科检查:全身皮肤密集分布大量绿豆至黄豆大紫红色结节,边界清楚,部分融合,按压不褪色,局部皮温不高,无触痛,表面无鳞屑及水疱,部分表面可见溃疡、结痂,皮损分布以四肢为主。实验室及辅助检查:血常规中WBC计数30.0×10^(9)/L,单核细胞计数5.9×10^(9)/L,血小板计数17×10^(9)/L,红细胞计数1.8×10^(12)/L,血红蛋白65 g/L。骨髓象示早幼粒细胞比值占1.5%,原始血细胞占4%,原始单核细胞占0%,幼稚单核细胞占6%,成熟单核细胞占10%,成熟淋巴细胞占20.5%。骨髓免疫分型示淋巴细胞占3.12%,粒细胞占58.68%。流式细胞术检查:髓系原始细胞比例增高(2.13%),CD45表达减弱,表型异常;粒系以中幼粒细胞及之后阶段为主;嗜酸性粒细胞易见,嗜碱性粒细胞比例增高;单核细胞占有核细胞的25.81%,比例增高;红系CD71表达减弱。染色体荧光原位杂交(FISH):BCR-ABL190及BCR-ABL210融合基因均阴性。染色体核型分析:46,XX(7)。腹部超声示脂肪肝,胆囊壁毛糙。皮损组织病理检查:表皮局灶破溃和结痂,真皮乳头水肿,真皮全层血管周围及胶原间可见巢团状单核细胞、组织细胞及散在的嗜酸性粒细胞浸润,可见胶原纤维。诊断:慢性粒单核细胞白血病并累及皮肤。治疗:给予静脉滴注地西他滨35 mg/d;化疗5 d后皮损逐渐消退。13个月后随访,患者已死亡,但皮损未复发。

A case of chronic myelomonocytic leukemia with diffuse,purplish red nodules as initial clinical manifestation is reported.A 75-year-old female presented with purplish red nodules and pruritus for 10 days.Dermatological examination showed numerous large purple nodules,mung beans to soybeans in sizes,with clear boundaries and focal ulceration,involving her entire body.Laboratory testing revealed that WBC count was 30.0×10^(9)/L,monocyte count was 5.9×10^(9)/L,platelet count was 17×10^(9)/L,red blood cell count was 1.8×10^(12)/L,and hemoglobin was 65 g/L.Bone marrow biopsy showed that the ratio of promyelocyte was1.5%,primitive blood cells were 4%,primitive monocytes were 0%,immature monocytes were 6%,mature monocytes were10%,and mature lymphocytes were 20.5%.Lymphocyte accounted for 3.12%,and granulocyte accounted for 58.68%.Flow cy tometry showed that the ratio of myeloid precursors was increased(2.13%),and the phenotype of the atypical infiltrate was abnormal with decreased CD45 expression.The granulocytes were mainly myelocytes with left shifted maturation.Eosinophilic granulocyte was easily seen,and the ratio of basophilic granulocyte was increased.Monocytes accounted for 25.81%of nucleated cells,and its ratio was increased.Erythroid CD71 expression decreased.Chromosome fluorescence in situ hybridization(FISH)analysis showed that the fusion genes of BCR-ABL190 and BCR-ABL210 were negative.Karyotype analysis revealed 46,XX(7).Abdominal ultrasound showed fatty liver and rough gallbladder wall.Histopathological examination of skin lesions showed focal epidermal ulceration,papillary dermal edema,atypical mononucleated cell infiltrate mixed with scattered eosinophils in the dermis around the blood vessels,and in between collagen bundles.A diagnosis of chronic myelomonocytic leukemia with skin involvement was made.The patient was treated with intravenous decitabine 35 mg/d chemotherapy for 5 days,and the skin lesions gradually subsided.At 13 months of follow-up after the treatment,the patient had died,but the lesions had not recurred.