Langerhans cell histiocytosis with digestive tract involvement

Gastrointestinal tract (GIT) involvement in Langerhans cell histiocytosis (LCH) is not commonly described. We present two children presenting with GIT involvement with LCH, one successfully treated on standard protocol and other being treated on a protocol for relapsed disease. A review of literature showed almost 95% children were less than 2 years of age and 62% were females. Vomiting, abdominal pain, constipation, intractable diarrhea, malabsorption, bloody stools, protein‐losing enteropathy, and even intestinal perforation are some of the reported symptoms. More than 50% patients died within 18 months from diagnosis. Pediatr Blood Cancer. 2010;55:748–753. © 2010 Wiley‐Liss, Inc.     

Liver involvement in Langerhans cell histiocytosis

Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient.     

A case of Langerhans cell histiocytosis presented with pneumothorax

Pneumothorax (PTX) is an unusual complication of Langerhans cell histiocytosis (LCH) in childhood. Spontaneous PTX is rare in childhood, and it is very rare in infancy. There are no specific recommendations for the treatment of PTX from LCH described in the literature. We are presenting a 19-month-old boy, who suddenly developed left-sided PTX with infiltrations in both lungs. He presented with PTX and skin lesions. He had a prolonged cardiac arrest, and although resuscitation was successful he required continuing ventilatory support (intermittent positive-pressure ventilation). Because he suddenly developed right-sided PTX and died on the second day of the admission, his LCH diagnosis was made only postmortem. So, he did not receive chemotherapy. It is likely that intermittent positive-pressure ventilation during the operation induced the development of much more multiple lung bullae, which subsequently ruptured, and/or it facilitated the development of the right-sided PTX. The patients with PTX and skin lesions, including babies, most likely have LCH and specific chemotherapy should be started in emergency, even before the final diagnosis is achieved.     

Langerhans cell histiocytosis with pulmonary involvement and unilateral pneumothorax

Langerhans cell histiocytosis (LCH) is a rare disorder of Langerhans cell with unknown etiology, which can uncommonly be associated with pneumothorax. A 14-month-old female is presented here who was referred to our center due to acute respiratory distress. Reticulonodular changes with multiple cystic areas were detected in chest X-ray, whilst extensive honeycombing and cystic changes were seen in high-resolution computed tomography scan. With deterioration of respiratory distress, chest X-ray was repeated, which revealed a unilateral pneumothorax. Meanwhile, some hyperpigmented skin plaques appeared on her skull and back. The biopsy results confirmed the diagnosis of proliferative histiocytosis. Prompt diagnosis of LCH and initiation of appropriate treatment in the patients who present with pneumothorax are vital to prevent further complications and even death in this group of patients.     

单一器官型郎格罕细胞组织细胞增生症一例

患儿男，3岁。因咳嗽、咳血发热10天住院。10天前因受凉后咳嗽，阵发性干咳，不伴咳痰和气喘。体温在38-39℃，间断咳血呈咖啡样伴有痰液，量约3ml左右。不伴皮疹与黑便。患儿第1胎第1产，足月顺产。新生儿期患左侧肺炎、胸腔积液住院20天治愈出院。无结核密切接触史。体检：体温37．4℃，脉搏118次／min，呼吸32次／min，体重8kg，血压70／50mmHg。营养发育差，精神萎靡，神志清，全身皮肤无黄染、瘀点瘀斑，未见皮疹与毛细血管扩张。贫血貌。左侧胸廓塌陷，左侧叩浊呼吸音低，无哕音，心脏与腹部     

儿童郎格罕细胞组织细胞增生症累及甲状腺一例

患儿男,3岁。因发现颈前肿物9个月入院。患儿于入院前9个月无意间发现颈前肿块,随吞咽可上下移动,无声音嘶哑、吞咽困难及疼痛等不适。肿块逐渐增大至鸡蛋黄大小。入院前8个月到当地医院就诊,诊断为“甲状腺恶性肿瘤伴肺肝转移”,给予中药(具体不详)口服,效果不明显。行胸部及腹部CT检查,肺及肝占位病变仍存在。于入院前1个月患儿无诱因出现发热,予抗炎治疗效果不佳。体检:一般情况可,皮肤未见包块及皮疹;扪及甲状腺左叶Ⅱ°肿大,约为2cm×1cm×7cm,未及结节,无压痛,未闻及血管     

朗格汉斯组织细胞增生症儿童肝脏受累的CT表现

目的：探讨朗格汉斯组织细胞增生症(LCH)儿童肝脏受累的CT表现。方法：回顾性分析9例经临床、实验室、病理检查确诊为LCH肝脏受累患儿的CT表现,其中男6例,女3例,全部病例均行肝脏CT平扫及动脉期、门脉期双期增强CT扫描。结果：9例患儿中, 主要CT表现为肝大（8例）;门静脉周围树枝状低密度灶及“门脉晕征”（7例）,增强后动脉期呈轻度至中度强化;肝内胆管扩张（5例）;肝门区及腹膜后淋巴结肿大（4例）;弥漫性低密度小结节灶（3例）,增强后呈环状强化。结论：LCH肝脏受累患儿CT表现具有一定的特点,了解这些特点有助于该病的早期诊断和治疗。     

Gastrointestinal tract involvement in Langerhans cell histiocytosis: case report and literature review

Digestive tract involvement in Langerhans cell histiocytosis is exceedingly rare. We report a case of Langerhans cell histiocytosis in an otherwise thriving neonate presenting with hematochezia, anemia, and rash. We also review the few cases of Langerhans cell histiocytosis with gastrointestinal involvement reported in the English-language medical literature. Although gastrointestinal involvement can range in severity from mild to life-threatening, its presence may be indicative of multisystemic disease, and aggressive treatment should be considered.     

Langerhans cell histiocytosis case with dense metaphyseal band sign

Eosinophilic granuloma, a type of Langerhans cell histiocytosis, exhibits a classic vertebral collapse, which is called vertebra plana (Calve's disease) and it manifests as a solitary bony lesion. Vertebra plana can cause severe pain in patients. Bisphosphonates (clodronate, pamidronate and zoledronic acid) have been recently used to treat osteolytic bone lesions of LCH. Zoledronic acid has 100 times relative potency that of pamidronate. We report a case of a 10‐year‐old girl who had zoledronic acid treatment for severe back pain due to vertebra plana. X‐ray photographs of the patient's body showed dense metaphyseal band sign, which can be found in lead poisoning, treated leukemia, healing rickets, recovery from scurvy, vitamin D hypervitaminosis, congenital hypothyroidism and hypoparathyroidism. Increased biological potent zoledronic acid deprived her of severe back pain due to vertebra plana and might cause dense metaphyseal band sign of her skeleton. Conclusion; We have cured the severe back pain of a 10‐year‐old girl case of eosinophilic granuloma with zoledronic acid. After that treatment, X‐ray photographs of the patient's body showed dense metaphyseal band sign. There have been few such cases reported until now.     

Thyroid involvement with Langerhans cell histiocytosis in a 3-year-old male

We report on the thyroid involvement with Langerhans cell histiocytosis (LCH) in a 3-year-old male. The patient presented with goiter and primary hypothyroidism. His goiter caused life-threatening airway obstruction. He developed locally invasive disease 4 years after his response to LCH therapy. LCH should be suspected as a cause of goiter and thyroidectomy is recommended.     

儿童多系统受累朗格罕细胞组织细胞增生症53例临床分析

目的分析多系统受累朗格罕细胞组织细胞增生症(MS-LCH)患儿的临床特征及远期预后,评价改良DAL-HX83/90方案对MS-LCH患儿的疗效。方法回顾性病例分析。研究对象为2011年1月至2019年5月郑州大学第一附属医院儿童医院血液肿瘤科收治的53例MS-LCH患儿,初始化疗采用改良DAL-HX83/90方案,按是否累及危险器官分为无危险器官受累(RO-)组和累及危险器官(RO+)组,RO+组再分为Ⅰ组(仅肺受累)、Ⅱ组(肺外,伴或不伴肺受累),总结临床特征和随访结果,Kaplan-Meier生存分析法计算生存率,Log-Rank检验及Cox比例风险回归模型对年龄、性别、危险器官受累、6周诱导化疗反应进行单因素及多因素预后分析。结果53例MS-LCH患儿中男34例、女19例,发病年龄21月龄(3月龄至13岁),RO-组31例,RO+组22例,其中Ⅰ组12例、Ⅱ组10例。随访时间51(12~144)个月,6周诱导化疗有效率89%(47/53),进展复发率30%(16/53),5年无事件生存率(EFS)为(67±6)%,5年总生存率(OS)为(83±5)%。单因素分析发现6周诱导化疗有效者5年EFS、OS明显高于无效者[(76±6)%比0,(88±4)%比(41±22)%],差异均有统计学意义(χ²=34.743、10.608,均P<0.05)。RO-组5年EFS、OS明显高于RO+组[(80±7)%比(49±10)%,(93±4)%比(70±10)%],差异均有统计学意义(χ²=6.022、4.793,均P<0.05)。Ⅰ组5年EFS明显高于Ⅱ组[(83±10)%比(10±9)%],差异有统计学意义(χ²=9.501,P=0.002),年龄、性别与EFS、OS无明显相关性(均P>0.05)。Cox比例风险回归模型分析发现6周诱导化疗反应是影响EFS(HR=13.114,95%CI 3.759~45.742,P<0.01)、OS(HR=7.748,95%CI 1.542~38.920,P=0.013)的独立危险因素。结论采用改良DAL-HX83/90方案治疗无危险器官受累MS-LCH,患儿多数可获长期生存。但累及肝、脾或造血系统的MS-LCH患儿疾病进展和复发率较高。     

Langerhans cell histiocytosis with involvement of nails and lungs in an adolescent

Skin and/or pulmonary involvement occur frequently in Langerhans cell histiocytosis (LCH) whereas nail involvement is rare. Herein, we present a case of LCH with initial nail disease and subsequent lung involvement causing recurrent pneumothoraces. Systemic chemotherapy was applied and intrapleural bleomycine and thoracoscopic pleurodesis with talc were performed. Although prognosis is not satisfactory in LCH with recurrent pneumothorax and nail involvement, the patient is under follow-up with no evidence of skin lesions, nail involvement, and pneumothorax for the last 10 months.     

联合免疫缺陷并郎格罕细胞组织细胞增生症一例

1 病历摘要 患儿,男,9个月,生后6个月无诱因出现反复高热、咳喘,伴前额枕部及颈部暗红色丘疹,边界清,无痒感,无腹泻,口腔黏膜反复出现乳白色附着物.查免疫球蛋白IgG1.82 g/L,IgA正常,结核菌素试验阴性,支原体抗体阴性,X线胸片示肺内炎症,胸、腹部CT示胸腺发育小,双肺、肝脏占位性病变,椎体附件、肋骨受累,提示系统性疾病,诊断不清,以"发热待查"收入院.患儿无特殊家族史,3个月和8个月时结核菌素试验均阴性.     

Langerhans cell histiocytosis mimicking molluscum contagiosum: A case series

Langerhans cell histiocytosis (LCH) is a disorder characterized by accumulation of Langerhans‐like cells in one or various organs. A correct staging work‐up is essential since there are multiorgan presentations with a poor prognosis. We report three patients with LCH skin lesions mimicking molluscum contagiosum in association with both high and low risk organ involvement. This peculiar cutaneous presentation can be a clue for the diagnosis of LCH, a disease with potentially severe systemic involvement.     

Congenital Langerhans cell histiocytosis with skin and lung involvement: Spontaneous regression

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare type of Langerhans Cell Histiocytosis, presenting at birth or during the neonatal period with eruption of multiple, discrete red-brown papules or nodules that undergo spontaneous regression. Systemic signs are generally absent. We describe a 4-month-old infant presenting with reddish brown nodular cutaneous lesions since birth with a past history suggestive of pulmonary involvement. Skin biopsy showed a dermal infiltrate of pleomorphic histiocytes; which were CD1a and S-100 positive, consistent with the diagnosis of CSHLCH. Both pulmonary and cutaneous lesions showed spontaneous resolution.     

Concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma

A 10‐year‐old girl with a family history of Hodgkin's lymphoma presented with a 2 month history of cervical lymphadenopathy and weight loss. Biopsy indicated concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma. Such an association is rare, especially so in children, but is not an isolated phenomenon, thereby prompting the question of whether Langerhans cell histiocytosis is a reactive or a neoplastic process.