江苏首见艾滋病合并马尔尼菲青霉病1例

目的报告1例江苏首见的艾滋病合并感染马尔尼菲青霉病。方法患者男,36岁。因持续高热至本院风湿科就诊。血、骨髓液肉汤增菌液37℃分离培养,ELISA法HIV(1+2型)抗体检测。流式细胞仪检测T淋巴细胞亚群。结果肉汤增菌液接种血培养基37℃进行培养,2天后血培养基上长出酵母样菌,25～28℃孵育下沙堡琼脂(SDA)培养基呈霉菌相,确诊为马尔尼菲青霉病。HIV(1+2型)抗体(+)。CD4/CD8<1.0,大肤康0.2g静滴1次/d,1周后热退,主动出院,失访。结论马尔尼菲青霉病是一种罕见的真菌病,临床医生对于发热、消瘦、贫血、淋巴结肿大、皮肤溃疡的艾滋病患者要警惕是否合并马尔尼菲青霉病,而对马尔尼菲青霉病要排除HIV感染的可能。     

A case of HIV infection complicated with penicilliosis marneffei

报告HIV感染并发皮肤播散性马尔尼菲青霉病1例.患者女,34岁.因持续发热40 d,全身散在分布丘疹就诊.皮损为全身散在分布暗红色丘疹,部分丘疹中央坏死、溃破,呈脐凹状.皮损真菌培养确诊为马尔尼菲青霉病,抗HIV抗体检测阳性.该文同时回顾了云南省报道马尔尼菲青霉病的文献.     

艾滋病并发皮肤播散性马尔尼菲青霉病

报告1例艾滋病并发皮肤播散性马尔尼菲青霉病.患者女,34岁.因间歇性发热、贫血4个月.面部及躯干丘疹、斑块1个月就诊.皮损表现为面部、躯干以及四肢广泛分布的疣状增殖性丘疹、斑块,上覆蛎壳样痂屑.皮损真菌培养及组织病理学检查确诊为马尔尼菲青霉病.经多次HIV抗体和抗原检测,最终确诊为艾滋病.予抗真菌及抗病毒治疗,患者皮损消退,一般情况迅速好转.     

AIDS并发马尔尼菲青霉及白念珠菌感染

报告1例AIDS并发马内菲青霉菌和白念珠菌感染。患者男,48岁。反复发热、进行性消瘦2个月,全身散在丘疹、脓疱、结节20余天。皮肤科检查见全身散在分布较多坏死性丘疹及部分传染性软疣样丘疹,颊黏膜及舌部附有大片白色凝乳样块状物。皮损组织病理检查示组织细胞内充满酵母样菌体,PAS染色阳性,并有大量葡萄状或桑椹状孢子聚集。皮损真菌培养见马内菲青霉菌生长。口腔分泌物真菌培养有马内菲青霉菌和白念珠菌生长。抗HIV抗体检测阳性。     

国内首例慢性播散性副球孢子菌病

报道国内首例慢性播散性副球孢子菌病。患者男, 49岁, 因皮肤丘疹、结节1年, 口腔黏膜丘疹、溃疡2个月入院。皮肤科检查:左足水肿, 左足底多发溃疡, 表面结痂, 左足第3、4趾间及第4、5趾间溃疡, 基底呈颗粒状, 伴点状出血、渗出;左足背、左足内侧及左膝多发丘疹、结节、斑块, 中央溃疡、结痂;左腕部2个丘疹, 上唇左侧1个丘疹, 表面结痂;牙龈、颊黏膜、唇黏膜及上颚可见红色斑块伴溃疡、点状出血, 皮损以左侧为主。浅表淋巴结彩超:双侧颈部及锁骨上窝淋巴结肿大, 左侧为著。胸腹部计算机断层扫描图像示:双肺弥漫粟粒样结节影及条索状、云絮状、结节状高密度影, 左侧肾上腺明显增粗。口腔、左下肢皮损组织真菌免疫荧光染色, 可见酵母细胞。口腔黏膜、左下肢皮损组织病理:肉芽肿性炎, 多核巨细胞内外可见酵母细胞, 折射双膜, 无芽、单芽或多芽;过碘酸希夫染色、六胺银染色阳性。左下肢皮损组织真菌培养:25 ℃、37 ℃沙氏葡萄糖琼脂培养基中培养阳性, 均为菌丝相。口腔黏膜及肺泡灌洗液宏基因组学测序:巴西副球孢子菌。诊断:慢性播散性副球孢子菌病。予伊曲康唑胶囊400 mg/d口服, 1个月后皮肤、黏膜皮...     

无皮肤损害的幼儿马尔尼菲青霉病2例

报告2例无皮肤损害的幼儿马尔尼菲青霉病。均为女性,分别为1岁2个月、1岁4个月。住所附近种植有竹子及甘蔗。临床表现为发热,未见皮肤损害、贫血、肝、脾肿大。骨穿示单核巨噬细胞胞浆内见成堆圆形或椭圆形的菌体。真菌培养25℃呈菌丝相,产生红葡萄酒色素,镜检见帚状枝;37℃呈酵母相,镜检见圆形、椭圆形、长形孢子。     

播散型Paget样网状细胞增生病国内首报

报告1例播散型Paget样网状细胞增生病。患者男,68岁。全身泛发浸润性斑块、脱屑30年,多发溃疡半年,伴发热。皮损组织病理检查示,表皮、真皮有异形淋巴样细胞浸润,类似Paget细胞。不典型淋巴样细胞免疫组化染色示CD45RO和CD3阳性。基因重排T细胞受体（TCR）-γ阳性。诊断：播散型Paget样网状细胞增生病。给予患者化疗,皮损迅速消退,但出现胸腔积液和腹水,腹腔淋巴结增大,最后死于多脏器衰竭。     

艾滋病并发马尔尼菲青霉病

报告1例艾滋病并发马尔尼菲青霉病。患者男,28岁。因消瘦、乏力3个月,面部和躯干丘疹及丘疱疹20 d就诊。皮肤科检查:躯干及四肢散在多个脐凹或传染性软疣样丘疹,口腔可见毛状假性白斑。皮损组织病理检查:表皮轻度增生,真皮内见多个肉芽肿,胞质内含有大量孢子。过碘酸希夫(PAS)染色(+),HIV抗体阳性;皮损和骨髓真菌培养示马尔尼菲青霉感染。诊断:艾滋病并发马尔尼菲青霉病。经抗真菌治疗后,全身皮损消退。     

艾滋病并发马尔尼菲青霉及白念珠菌感染

报告1例艾滋病并发马尔尼菲青霉及白念珠菌感染.患者男,21岁.发热、消瘦6个月,全身起丘疹、结节2个月.皮肤科检查:全身尤其面部分布较多丘疹及软疣样突起,颊黏膜及舌部有大片白色凝乳样块状物.皮损组织病理检查:组织细胞内充满酵母样菌体,PAS染色阳性,呈葡萄状或桑椹状聚集,个别孢子两头钝圆,中间有横隔.皮损真菌培养见马尔尼菲青霉生长,口腔分泌物真菌培养见马尔尼菲青霉和白念珠菌生长.抗HIV抗体检测阳性.     

马尔尼菲青霉菌病1例实验诊断报告

目的介绍1例艾滋病合并播散性马尔尼菲青霉菌病的实验诊断,探讨该菌的培养、鉴定特点。方法通过真菌直接镜检、大培养、小培养、组织病理学检查和染色观察。结果马尔尼菲青霉菌早期培养菌落比较典型。晚期菌落颜色变化较多。37℃培养,镜下多呈鹿角样或杆状的粗短菌丝。涂片、组织学检查易与其他青霉菌、内脏利氏曼小体、组织胞浆菌等混淆。结论马尔尼菲青霉菌病诊断的"金标准"仍是真菌培养鉴定。     

A Rare Case of Cutaneous T-Cell Lymphoma Accompanied by Acute Monoblastic Leukemia and Diffuse Large B-Cell Lymphoma

A 70-year-old female was referred for brown-to-gray colored papules and nodules on her lower legs. She had been diagnosed with diffuse large B-cell lymphoma (DLBCL) in her stomach, and myelodysplastic syndrome (MDS) by bone marrow biopsy. Three years after complete remission of DLBCL, she experienced DLBCL recurrence in her small bowel and was hospitalized. MDS had been stationary, but during the treatment of DLBCL, her laboratory findings suggested signs of leukemia. Bone marrow biopsy was done, and acute monoblastic leukemia (AMoL) was diagnosed. After 1 cycle of chemotherapy for AMoL, skin lesions developed, and her skin biopsy showed cutaneous T-cell lymphoma (CTCL). Terminal deoxynucleotidyl transferase staining and CD123 staining were negative, and bone marrow re-biopsy conducted after the skin lesion developed still showed monoblastic proliferation. Whether the CTCL represented with an AMoL lineage switch could not be completely proved due to the absence of molecular or clonal marker evaluations, but the possibility of coexistence of three different malignancies was higher. During treatment, a neutropenic fever developed, and the patient died due to sepsis. We herein report a rare case of CTCL accompanied by AmoL and DLBCL.     

艾滋病合并马尔尼菲青霉病11例临床分析

目的 探讨11例艾滋病合并马尔尼菲青霉病患者的临床特征.方法 对2002-2004年来我院住院和门诊就诊的11例艾滋病合并马尔尼菲青霉病患者的一般资料、临床表现、体征、实验室检查进行分析.结果 11例患者中男9例,女2例,临床症状除非特征性的表现如发热、消瘦、贫血、肝脾淋巴结肿大外,8例合并有皮疹;3例有消化道症状,其中2例腹痛,1例腹泻、排红色湖状便:1例吞咽困难,进食梗阻感;5例有呼吸系统症状;1例关节痛;2例合并口腔念珠菌感染、4例骨髓、1例痰、1例血涂片、8例皮疹刮片或分泌物涂片、2例淋巴结和皮肤病理活检切片中可见成堆的PAS染色阳性的圆形或腊肠形分隔孢子.11株分离株经形态学、培养皆证实为马尔尼菲青霉,其中4株分离株经DNA分析证实为马尔尼菲青霉.结论 艾滋病患者机会性感染中马尔尼菲青霉病的临床表现多样,且多伴有皮疹.     

Malignant histiocytosis with panniculitis--a case report

We report a case of malignant histiocytosis which began with the skin lesions of panniculitis. A 32-year-old woman presented with recurrent erythematous plaques, subcutaneous nodules, and ulcers on the trunk and the extremities and intermittent fever for 7 months. The cutaneous lesions consisted of erythematous and brownish irregular-shaped patches and tender cutaneous nodules 0.5-1.0 cm in diameter. Central necrosis and shallow ulcers were seen in the lesions. The patient also suffered from general fatigue, arthralgia, and weight loss. She was anemic and thrombocytopenic and had progressive impairment of liver function with coagulation defect. Histopathological study of skin lesions showed lobular panniculitis without vasculitis in the subcutaneous fat tissue. In the panniculitis lesion, moderate mixed cell infiltration consisting of lymphocytes and histiocytes was observed. Bone marrow aspiration revealed an increase in the number of histiocytes, mostly immature with active phagocytosis of erythroid cells, myeloid cells, and platelets. She was diagnosed as having malignant histiocytosis and treated with cyclophosphamide, vincristine, and prednisolone which she responded well; her fever subsided and the lesions healed with hyperpigmentation. In this patient, benign histiocytes with hemophagocytosis without immature forms were found in the skin lesions. According to our knowledge, this is the first Thai report of malignant histiocytosis with clinical features of panniculitis.     

儿童皮下脂膜炎样T细胞淋巴瘤合并嗜血细胞综合征一例附文献复习

患儿,女,8岁。皮肤结节、斑块8个月,右大腿肿胀1个月,伴发热1周。肝脏肋下约1~2 cm可触及、脾脏肋下约2~3 cm可触及,质软,无触痛。实验室检查:血细胞降低,肝酶、血脂、乳酸脱氢酶、C反应蛋白升高,血沉加快,凝血功能异常,铁蛋白升高。免疫组化:CD3、CD8、颗粒酶B、βF1阳性,Ki-67:约50%(+)。骨髓活检:骨髓增生活跃。皮损病理检查:皮下脂肪见异型细胞。诊断为脂膜炎样T细胞淋巴瘤合并嗜血细胞综合征。我们对相关文献进行了复习。     

A case of subcutaneous panniculitis-like T-cell lymphoma with haemophagocytosis developing secondary to chemotherapy

A 45-year-old woman presented with fever, generalized skin lesions and multiple lymphadenopathies. In her past history she had had six courses of cyclophosphamide and cisplatin combination chemotherapy 7 years ago because of an ovarian carcinoma. We found pancytopenia in the peripheral blood examination. Skin biopsy showed diffuse subcutaneous infiltration reminiscent of panniculitis but composed of malignant lymphoid cells that were of T lineage. Bone marrow biopsy showed normocellular myeloid tissue with abundant haemophagocytic macrophages. Subcutaneous panniculitis-like T-cell lymphoma with haemophagocytic syndrome was diagnosed. This is the first case reported of subcutaneous panniculitis-like lymphoma occurring secondary to chemotherapy.     

Skin infiltration of juvenile myelomonocytic leukemia

We described a two-year-old boy who developed a skin infiltration from JMML. Several indurated erythematous lesions were seen on his back on his first visit to our department. Edematous erythemas had repeatedly appeared on his auricles and feet for the previous six months. He had had a high fever for a month. Hepatosplenomegaly and superficial lymphadenopathy were recognized. Laboratory investigation showed leukocytosis and anemia. The diagnosis of JMML was confirmed by the findings of myeloid hyperplasia in his bone marrow and the spontaneous colony formation and GM-CSF hypersensitivity in a culture of bone marrow cells. Histopathologically, large atypical mononuclear cells were infiltrated throughout the dermis in a perivascular and interstitial distribution in a skin biopsy specimen. These cells were CD3 (-), CD20 (-), CD45 (+), CD68 (+) and myeloperoxidase (+). Bone marrow transplantation and then cord blood stem cell transplantation were performed but soon rejected. The indurated erythematous lesions appeared again soon after the relapse of JMML. There are other reported cases of JMML with skin infiltration that preceded any other manifestations of the disease. JMML cells in some patients, including our case, seem to have a great affinity for the skin, and skin biopsy aids in early detection of this disease.     

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.     

Bone marrow examination has limited value in the staging of patients with an anaplastic large cell lymphoma first presenting in the skin. Retrospective analysis of 107 patients

Background According to criteria of the World Health Organization–European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas a diagnosis of primary cutaneous CD30‐positive anaplastic large cell lymphoma (C‐ALCL) should be made only when systemic localizations have been excluded by adequate staging procedures, including a bone marrow biopsy. It has recently been questioned whether or not bone marrow examination should be performed routinely in indolent cutaneous lymphomas such as C‐ALCL. Studies addressing this issue have never been performed. Objectives To determine the incidence of bone marrow involvement in patients with an ALCL first presenting in the skin to find out if the current policy to advise bone marrow examination should be maintained or whether a bone marrow biopsy should be performed only in selected cases. Methods All patients presenting with skin lesions with histological and immunophenotypical features of an ALCL were retrieved from the database of the Dutch Cutaneous Lymphoma Group. Patients with a history of systemic ALCL and patients without bone marrow examination were excluded from the study. The final study group included 107 patients with an ALCL first presenting in the skin, who had been staged completely. Results Staging procedures showed the presence of extracutaneous disease in 20 patients, but bone marrow involvement was not detected in any of the 107 patients. Moreover, only one patient developed bone marrow involvement during follow up (median follow‐up period 69 months). Conclusions Bone marrow examination has limited value in the staging of patients with an ALCL first presenting in the skin, and should be performed only in selected cases.