急性白血病骨髓移植术后并发皮肤髓系肉瘤

患者男，22岁。因急性白血病行造血干细胞移植术后3年，左大腿单发红色斑块2个月。皮肤科检查：左大腿伸侧可见一红色半球形斑块，质稍韧，活动度较差，大小约5 cm×5 cm，边界清，其上可见白色鳞屑及黄痂。皮损组织病理检查：真皮全层可见瘤细胞弥漫性浸润，瘤细胞呈中等大小，圆形至卵圆形，可见有凹痕的肾形嗜碱性的细胞核，偶尔也可见大的异形细胞。免疫组化：溶菌酶（+），CD43(+),CD99(+),CD68(+),CD15部分（+），髓过氧化物酶（-），CD3(-),CD20(-),CD30(-),CD56（-）。诊断：急性白血病（M5）骨髓移植术后；皮肤髓系肉瘤。     

急性髓细胞性白血病(M4型)

报告1例急性髓细胞性皮肤白血病(M4型).患者女,48岁.全身出现丘疹、红色结节14d,伴剧烈瘙痒.体格检查:全身泛发大小不等的红色丘疹、结节,质韧,无压痛.皮损组织病理检查:真皮内弥漫淋巴样细胞浸润,有明显异形及较多核分裂象.免疫组化组织病理检查:CD68阳性(灶性),MPO阳性(少量).骨髓穿刺:白血病细胞大量增生,免疫标记:CD68、CD11b、MPO及HLA-DR均阳性.诊断:急性髓细胞性白血病(M4型).患者经过2次DA(伊达比星、阿糖胞苷)方案化疗后,再次行骨髓穿刺示缓解,但皮损仍有复发.     

以急性发热性嗜中性皮病为首发症状的急性髓系白血病1例

 报告以急性发热性嗜中性皮病为首发症状的急性髓系白血病1例。患者女,50岁,反复全身红色斑块伴疼痛4个月,加重2 d。皮肤科检查：颈部及双上肢分布大小不等红色斑块,皮损边缘略隆起于皮面,呈环状,未见水疱及破溃,触痛阳性。上唇少许糜烂面。组织病理符合急性发热性嗜中性皮病改变。入院后查血液系统异常,诊断急性髓系白血病,以急性发热性嗜中性皮病为首发症状。予EA方案预化疗。三个月后患者因严重脑出血死亡     

寻常性银屑病皮损处神经纤维的数量及其与朗格汉斯细胞关系的观察

目的 观察寻常性银屑病患者皮损处神经纤维的数量变化及其与朗格汉斯细胞的关系.方法采用免疫组化过氧化物酶法观察28例寻常性银屑病患者皮损处神经纤维的数量变化;采用免疫荧光双标记及共聚焦激光扫描显微镜技术观察寻常性银屑病患者皮损处神经纤维与朗格汉斯细胞的关系.结果寻常性银屑病患者皮损处神经纤维长度显著增加,与正常人对照比较有统计学差异(t=4.09,P＜0.001).皮损表皮内神经纤维与朗格汉斯细胞的接触明显增多,与正常人对照比较有统计学差异(t=3.55,P＜0.01).结论寻常性银屑病皮损处神经纤维长度显著增加,表明此部位神经纤维增生明显,并且表皮内神经纤维与朗格汉斯细胞的直接接触明显增加,提示银屑病患者神经系统的免疫调控作用可能是通过改变朗格汉斯细胞的功能实现的。     

长期以头部皮肤损害为表现的急性髓细胞性白血病1例

报告1例长期以头部皮肤损害为表现的急性髓性白血病(AML)并分析皮肤损害特征、组织病理及髓过氧化物酶(MPO)免疫组化染色特点。该患者为男性,63岁,以局限性头部皮损反复发作7月为其主要症状。皮损呈多形性,以红斑、脓疱、糜烂及溃疡为主,短期内四肢出现浸润性斑块。骨髓病理及流式细胞术提示急性髓性白血病M2型。头部皮肤组织病理见血管周围散在的肿瘤细胞,表达MPO阳性。白血病临床表现复杂,头部浸润是其皮肤受累部位之一。在白血病的诊断以前出现头部皮疹,活检提示有异型细胞浸润,提示白血病的进展及预后不良。     

严重银屑病在同种异体骨髓移植后病损完全消失

患者男,36岁,有20年严重的、难治性银屑病病史,接受过补骨脂素、UVA及氨甲喋呤等多种治疗,以期控制银屑病.1984年11月患急性粒-单核细胞型白血病,经阿糖胞苷、柔红霉素、硫鸟嘌呤联合化疗而迅速缓解,但严重银屑病继续存在,需用氨甲喋呤治疗.1985年8月为准备接受骨髓移植,应用2次环磷酰胺(每次60mg/kg),并以14Gy作全身照射(分7次照射).而后接受其胞兄的骨髓(以单克隆抗体MBG6、RFT8消耗供髓中的T细胞).骨髓移植后,银屑病皮损即完全消失,尽管停用细胞毒和免疫抑制治疗,但银屑病未复发.周围血淋巴细胞DNA分型证实为嵌合体,具有供体的DNA型.     

急性髓细胞性白血病M2型所致皮肤白血病一例

报道1 例急性髓细胞性皮肤白血病.患者男,38岁,因确诊急性粒细胞性白血病1年,躯干部皮疹1个月入院.皮肤科检查:躯干、右额部皮肤散在数十个直径约1～2 cm大小的红色结节及斑块,略高出皮面,触之有明显浸润感,无压痛.骨髓检查及免疫学证实为急性髓细胞性白血病M2型,患病1年后躯干及前额皮肤出现斑块及结节,皮肤组织病理及免疫组化证实为皮肤白血病.白血病短期内出现皮肤、肺、脾、肾、胸腰骶椎多发性浸润,则提示病情急性进展及预后不良.     

FoxM1在寻常性银屑病患者皮损中的表达

目的研究寻常性银屑病患者皮损中Fox M1(forkhead box M1)表达情况。方法寻常性银屑病及正常对照标本各30例,采用En Vision二步法进行免疫组化染色。结果 Fox M1在正常皮肤组织中表达减低,在寻常性银屑病皮损染色强度显著增高(Z=-4.965,P=0.000,P0.05)。结论 Fox M1可能与银屑病皮损角质形成细胞过度增生相关。     

皮肤粒细胞肉瘤

报告1例皮肤粒细胞肉瘤.患者男,50 岁.3 年前不慎被竹片刺伤,皮损处出现结节,逐渐增大、增多,沿淋巴管方向分布于双上肢.曾行外周血常规及骨髓穿刺检查均正常.入院行组织病理检查示低分化髓细胞髓外(皮肤和皮下脂肪组织)浸润.免疫病理示髓过氧化物酶(MPO)(++)、Ki67(++)、CD43(+).组织病理诊断为皮肤粒细胞肉瘤.确诊后行骨髓穿刺提示急性粒细胞白血病(AML,M2).转血液科接受化疗,化疗无效很快死亡.     

寻常性银屑病患者皮损白介素17、干扰素γ及巨噬细胞炎性蛋白3α mRNA的表达

目的 探讨寻常性银屑病患者皮损中白介素17(IL-17)、干扰素γ(IFN-γ)以及巨噬细胞炎性蛋白3α(MIP-3α)mRNA的表达与发病的关系.方法用逆转录-聚合酶链反应(RT-PCR)方法检测了31例寻常性银屑病患者皮损和16例正常人皮肤中IL-17、IFN-γ和MIP-3αmRNA的表达.结果寻常性银屑病患者皮损及正常人皮肤组织中均有IL-17、IFN-γ和MIP-3α mRNA的表达.在寻常性银屑病患者皮损中IL-17 mRNA的表达水平为1.142±0.059(A值,下同),较正常人对照组(0.879±0.034)明显增高(P＜0.001);IFN-γ mRNA的表达水平在皮损和正常人对照分别为1.114±0.056和0.905±0.026,差异有显著性(P＜0.001);MIP-3αmRNA的表达水平为1.140±0.052,亦明显高于正常人对照组(0.868±0.031)(P＜0.001).结论寻常性银屑病患者皮损中IL-17、IFN-γ及MIP-3αmRNA表达水平的上调可能与银屑病的发病相关。     

Congenital leukemia cutis

Abstract:  We describe a premature neonate who was born with pancytopenia and a single subcutaneous nodule on her right lower extremity. A biopsy specimen from the nodule demonstrated a dense infiltrate of pleomorphic mononuclear cells that extended throughout the dermis and into the subcutaneous tissue. Immunohistochemical stains and bone marrow examination confirmed a diagnosis of acute myelogenous leukemia. Cytogenetic studies on peripheral blood by G-banding analysis revealed an abnormal karyotype of 46, XX, ins[inv(10)(p11.2q22.2);11](q22.2;q13.2q23.2). A split in the mixed lineage leukemia gene was identified by fluorescence in situ hybridization. Induction chemotherapy was started but was complicated by multiorgan failure. The patient died on the eleventh day of life. As leukemia cutis more typically presents as multiple infiltrative papules, nodules, or plaques, we stress the importance of including leukemia in the differential diagnosis of a solitary nodule in a neonate.     

Follicular Mucinosis in a Male Adolescent with a History of Acute Myelogenous Leukemia and Graft‐versus‐Host Disease

Although many cases of follicular mucinosis are idiopathic, numerous others are associated with mycosis fungoides or, rarely, other neoplastic or inflammatory disorders. There are only three reported cases, all in adults, of follicular mucinosis arising in association with acute myelogenous leukemia, two of which involved mycosis fungoides–associated follicular mucinosis, including one case in which the patient had a preceding bone marrow transplant. We present the first reported case of follicular mucinosis arising in an adolescent with acute myelogenous leukemia and acute graft‐versus‐host disease after an allogeneic bone marrow transplantation.     

Leucemia cutánea en un paciente con leucemia mieloide aguda M2

—The leukemia cutis is the cutaneous infiltration by leukemics cells. Patients with acute myelogenous leukemia (AML) present specific cutaneous involvement in approximately 10% of the cases.We report the case of a leukemia cutis in a 73-year-old male with AML-M2. The patient presented with a one-week history of general malaise, asthenia, cough, dysnea and fever. Physical examination revealed and indurated red-brown plaque on his back of two moths duration, which was pruritic. Concomitance blood and bone marrow findings were diagnostic of AML-M2. A cutaneous biopsy was consistent with leukemia cutis. A CT and a bronchial biopsy showed pulmonary involvement. Since starting chemotherapy the patient had complete remission and the cutaneous lesion cleared, but 15 days later the skin lesion reappeared. A new bone marrow examination revealed recurrent leukemia. He died one month later.     

The spectrum of cutaneous disease in leukemias

Cutaneous eruptions are frequent complications in the clinical course of patients with leukemia. Leukemia cutis is occasionally the cause of the eruption, but in many cases the lesions are non-leukemic. We have retrospectively selected all skin biopsies from patients with a computer-coded diagnosis of leukemia seen in the Stanford University Department of Pathology in the last 7 years, and separated these cases into the broad categories of acute myelogenous leukemia (AML), acute lymphocytic leukemia (ALL), chronic myelogenous leukemia (CML), and chronic lymphocytic leukemia (CLL). We also analyzed separately those cases seen in patients treated with bone marrow transplantation from those treated with standard chemotherapy regimens. We found that leukemia cutis was seen frequently as the cause of lesions in patients with CML and CLL. In contrast, a wide variety of lesions were seen in patients with AML, including a greater number of infectious lesions, drug reactions, vasculitis, and lesions secondary to a hemorrhagic diathesis. In the bone marrow transplantation patients, graft vs host disease was usually the cause of skin lesions in those transplanted for CML and ALL, but again those patients with an underlying diagnosis of AML showed a wide variety of lesions including drug reactions, fungal infections and leukemia cutis. Finally, 6% of cases from patients with AML showed intraepidermal blistering disorders of various types, an association that has not been previously reported.     

Staphylococcal scalded skin syndrome mimicking acute graft-vs-host disease in a bone marrow transplant recipient

A 33-year-old man with mild acute graft-vs-host disease after an allogeneic bone marrow transplant for chronic myelogenous leukemia developed a necrolytic rash 90 days after transplant. A diagnosis of staphylococcal scalded skin syndrome was made when a skin biopsy specimen revealed a split in the granular layer and phage group 2, type 71 Staphylococcus aureus was cultured from the blood.     

皮肤白血病1例

患者，男性，22岁。1999年5月出现发热，经血液和骨髓穿刺证实为急性骨髓性白血病M5型（急性单核细胞性白血病）。2000年2月行自身外周血干细胞移植并顺利植活。移植后4个月患者发现前胸部出现紫红色结节、斑块，组织病理为“继发皮肤白血病”，骨髓穿刺和皮损免疫组化诊断为“急性骨髓白血病M5型复发”。经10天化疗，患者皮损减轻。     

Spontaneous regression of aleukemia congenital leukemia cutis

A full-term 2-week-old boy was referred to the pediatric dermatology clinic with numerous blue to violaceous nodules present since birth. TORCH titers (against toxoplasmosis, cytomegalovirus, herpes simplex virus, rubella, and syphilis) were negative. Complete blood count and peripheral smear were normal. A skin biopsy specimen showed an atypical cellular infiltrate suspicious for leukemia or lymphoma. A bone marrow biopsy specimen demonstrated acute myelogenous leukemia (M4 subtype). Following consultation with pediatric oncology and the recognition of the potential for spontaneous regression, chemotherapy for the infant's condition was not recommended. He remained otherwise healthy and was followed-up with biweekly to monthly complete blood counts and physical examinations, which were repeatedly normal. By 3 months of age, the nodules had completely resolved and there was no evidence of recurrence at 8 months of follow-up. We report this instance of aleukemic congenital leukemia with spontaneous regression of leukemia cutis without therapeutic intervention.     

Dermatologic changes associated with roquinimex immunotherapy after autologous bone marrow transplant

BACKGROUND: Roquinimex (Linomide) is an immunotherapeutic agent used in conjunction with autologous bone marrow transplantation (ABMT) for treatment of acute and chronic myelogenous leukemia (AML and CML). This agent may induce graft-versus-host reactions (GVHR) as well as graft-versus-leukemia (GVL) effects. OBJECTIVE: We documented the incidence of acute cutaneous GVHR associated with roquinimex immunotherapy. The presence or absence of autologous GVHR was also correlated with a potential GVL effect in patients with CML treated with ABMT and subsequent roquinimex immunotherapy in the period after the transplant. METHODS: Fifteen patients undergoing bone marrow transplantation and roquinimex immunotherapy for CML were followed up, and clinicopathologic data were analyzed. RESULTS: Acute cutaneous GVHRs were observed in 6 of 15 patients (40%) treated with roquinimex. Ten of 11 evaluable patients receiving roquinimex exhibited eccrine sweat gland necrosis (ESGN) (90.9%), which was independent of the acute GVHR. Neither bone marrow engraftment status nor the survival rates of patients with and without GVHR was significantly different. CONCLUSION: Roquinimex immunotherapy enhances the incidence of GVHR and was associated with a high rate of ESGN in patients with CML who were undergoing ABMT. There was no significant association between ESGN and acute GVHR. Acute autologous GVHR caused by roquinimex did not correlate with a GVL effect in our study of 15 patients with CML.     

Sweet's syndrome masquerading as facial cellulitis

Sweet's syndrome, or acute febrile neutrophilic dermatosis, is a cutaneous condition that typically occurs as tender red plaques or nodules. However, atypical presentations may occur and, in our case, Sweet's syndrome masqueraded as facial cellulitis and soft tissue infections of the extremities in a sporotrichoid pattern. Despite treatment with broad-spectrum antibiotics, the cutaneous lesions progressed. Results of skin biopsy specimens of the facial plaque and a nodule on the right upper extremity were diagnostic of Sweet's syndrome. Simultaneous to diagnosis, the patient also was found to have acute myelogenous leukemia (AML).     

Myeloid sarcoma in a newborn: A rare manifestation of congenital acute myeloid leukemia

Cutaneous myeloid sarcoma is rarely present prior to the diagnosis of congenital acute myeloid leukemia (AML); the former is typically diagnosed with or after the leukemia. We report a 2-day-old male born with multiple cutaneous red to violaceous nodules. Histopathologic and immunohistochemistry findings from a skin nodule were suspicious for myeloid sarcoma. Bone marrow biopsy was initially negative for aberrant blasts; however, at age 4 months, AML with a KMT2A gene rearrangement was identified via bone marrow biopsy.