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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of congenital systemic Langerhans cell histiocytosis (LCH), diagnosed and treated in our department from June 1995 until May 1996, are described. The cases concern two neonates (one female and one male) born with necrotic lesions and skin nodules. The diagnosis was confirmed by skin biopsy which showed diffuse infiltration by CD1 antigen and S-100 protein positive histiocytes. The babies didn't present with anemia, hepatosplenomegaly or lymphadenopathy. Hepatic and renal function were normal. In both infants skeletal survey showed no lytic lesions but chest X-rays and high resolution computerized tomography (HRCT) scan revealed diffuse mottling of both lung fields. Bone marrow aspiration showed the presence of histiocytes in percentages of 6% and 10%, respectively. Both babies were treated with prednisolone 1 mg/kg body weight for three months. The first child who is 20 months old, is now well with resolution of skin and pulmonary lesions occurring within one month of the initiation of steroids, while the second, who presented spectacular resolution of skin lesions within the first three weeks of therapy, is also in excellent condition five months after completion of treatment. We conclude that congenital LCH has to be suspected in neonates with persisting skin lesions. If the disease is systemic but without organ dysfunction, treatment with steroids may be beneficial.
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PMID:Congenital systemic Langerhans cell histiocytosis (report of two cases). 940 65

We report a 74-year-old man who presented with a rash on the trunk showing clinical and histological features of non-Langerhans cell histiocytosis. Two years after presentation he developed weight loss, lymphadenopathy and hepatosplenomegaly; a diagnosis of lymphocyte-predominant Hodgkin's disease was made on lymph node biopsy. The cutaneous signs and lymphoma responded to chemotherapy. Taken in conjunction with previously published reports of associations between cutaneous histiocytoses and haematological malignancies, we recommend close observation of patients in whom a diagnosis of non-Langerhans cell histiocytosis is made.
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PMID:Non-Langerhans cell histiocytosis associated with lymphocyte-predominant Hodgkin's disease. 1056 22

Langerhans cell histiocytosis (LCH) and Niemann-Pick disease are very rare diseases. Both LCH and Niemann-Pick disease are distinguished by complex etiology, pathogenesis and broad spectrum of clinical manifestations. In our study we presented case report of 2.5 year old girl in who the coexistence of both diseases was noted. General symptoms that led to diagnosis were: hepatosplenomegaly, cholestatic jaundice, cutaneous lesions and mental development retardation.
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PMID:[Coexistence of Langerhans cell histiocytosis and Niemann-Pick disease in children: a case report]. 1073 91

Langerhans cell histiocytosis is a rare pathology. It may be a single system disease at a single site or at multiple sites, or a multisystem disease; it may evolve from one of these forms into another. Oral manifestations may be the first signs of the disease. Little is known as yet about its etiology. We review the nosological entity and present the case of a two-year-old boy suffering from the multisystem form, paying particular attention to the oral manifestations. Physical examination on admittance revealed skin lesions in the scalp, armpits and groins, hepatosplenomegaly and bilateral otorrhea. The diagnosis was multisystem Langerhans cell histiocytosis, affecting the skin, bone, liver and CNS.
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PMID:Oral manifestations of Langerhans cell histiocytosis. Case study of a two-year-old boy. 1255 19

Juvenile xanthogranuloma (JXG), one of the most common forms of non-Langerhans cell histiocytosis (LCH), usually presents in young children as spontaneously regressing cutaneous lesions. However, the systemic type of JXG is difficult to treat in newborn infants, and fatal cases have been reported. In the patient described here, solid masses were discovered by fetal sonography during the 38th gestational week. At birth she had multiple tumors on the back, cheek, and hip as well as marked hepatosplenomegaly accompanied by respiratory failure. Laboratory results indicated pancytopenia, obstructive liver dysfunction, and coagulopathy. Brain magnetic resonance imaging revealed a tumor at the left pontine angle, and dysmorphic histiocytes were present in her spinal fluid. She was diagnosed with systemic JXG by histopathologic findings of the hip mass. The LCH-based multiagent chemotherapy including cytarabine, vincristine, methotrexate, and prednisolone ameliorated the symptoms rapidly. She was treated for 12 months and is currently doing well as a normally developing 2-year-old.
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PMID:Successful treatment of congenital systemic juvenile xanthogranuloma with Langerhans cell histiocytosis-based chemotherapy. 1516 50

Bacillus Calmette Guerin (BCG) lymphadenitis is a well known entity. Disseminated BCG infection usually presents as generalized lymphadenopathy, skin rash and hepatosplenomegaly and at times, can pose a diagnostic challenge to clinicians. There are only a few published studies on the cytological findings of BCG lymphadenitis. In this letter we report the fine needle aspiration cytology (FNAC) of BCG lymphadenitis clinically masquerading as Langerhans cell histiocytosis (LCH). FNA smears showed sheets of foamy macrophages and many polymorphs in a dirty necrotic background with many macrophages as well as polymorphs showing negatively stained rod like structures within their cytoplasm. Zeihl Neelson stain revealed that these cells were heavily loaded with acid fast bacilli (AFB). In the index case, AFB were also seen within the cytoplasm of polymorphs, which has not been documented earlier in the literature.
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PMID:FNAC of Bacillus- Calmette- Guerin lymphadenitis masquerading as Langerhans cell histiocytosis. 1561 35

We report a case of juvenile xanthogranuloma (JXG) having progressive pancytopenia for 6 months until the proliferating skin lesions. A 2-month-old infant presented recurrent fever, anemia, and hepatosplenomegaly mimicking hemophagocytic lymphohistiocytosis (HLH) or juvenile myelomonocytic leukemia (JMML). At 8 months of age, the biopsy of a growing papule on the elbow made the diagnosis. Bone marrow (BM) specimens showed clustering foamy cells including hemophagocytosis by histiocytes. Treatment with etoposide followed by vinblastine plus prednisolone (PSL) therapy improved the disease. Although JXG is a benign non-Langerhans cell histiocytosis, the multisystem-visceral form should be considered as a potential aggressive disease when associated with BM failure in early infancy.
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PMID:Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma. 1604 46

An 8-month-old male infant presented with a progressively worsening generalized rash of 5-6 months duration, fever, poor feeding, and abdominal distension. An initial laboratory workup revealed anemia, thrombocytopenia, and hepatosplenomegaly. The patient was started on i.v. antibiotics, and a working diagnosis of Langerhans cell histiocytosis was reached that was later confirmed with a skin biopsy. Subsequently, the patient received first-round chemotherapy with vinblastine and prednisone, on which he appeared to improve clinically; however, he soon relapsed. He then received combination salvage therapy with cladribine (2CdA) and cytarabine (Ara-C) for three cycles. The patient responded well to this regimen with resolution of his condition. The patient was then referred for a bone marrow transplant.
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PMID:Langerhans cell histiocytosis, a case of Letterer Siwe disease. 1978 12

A full-term female baby was admitted to our hospital at the postnatal age of 37 days with generalized vesiculopapular, crateriform skin lesions. Physical examination revealed a well-nourished baby without fever, hepatosplenomegaly or lymphadenopathy. Laboratory examination was normal except for thrombocytosis (platelet count, 970 x 10(3)/microL). All studies for herpes simplex virus, including culture, polymerase chain reaction and IgM, were negative except for an antigen test from the vesicles for herpes simplex virus type 1, which was positive. Chest X-ray showed increased reticulogranular infiltration over bilateral lung fields and some osteolytic lesions at the left parietal bone. Skin biopsy revealed infiltration of Langerhans cells and eosinophils, plus positive CD1a and S-100 stains. The diagnosis was reconfirmed by a second hospital and chemotherapy was given. In this case report, the differential diagnoses of neonatal vesiculopapular skin lesions, and the classification and outcome of neonatal Langerhans cell histiocytosis are presented.
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PMID:Langerhans cell histiocytosis in a newborn. 1994 41

The development of xanthogranulomas has been linked to hematologic malignancies in children and adults, based on a number of reports in the literature. In children, a specific association between juvenile xanthogranuloma, neurofibromatosis 1, and juvenile myelomonocytic leukemia has been described. We report a case of a 9-month-old child, without a known diagnosis of neurofibromatosis 1, who presented with hepatosplenomegaly, anemia, thrombocytopenia, and multiple cutaneous nodules, which were confirmed to be juvenile xanthogranulomas upon biopsy. A concurrent work-up showed that the child had juvenile myelomonocytic leukemia. Although cutaneous juvenile xanthogranulomas are benign lesions, in several reported cases they have been shown to herald leukemia. This association between xanthogranulomas and hematologic malignancy is poorly understood. Juvenile xanthogranulomas have a number of morphologic variants and clinical presentations that can be confused with the cutaneous lesions of Langerhans cell histiocytosis and dermatofibroma. Recognition of the broad clinicopathologic spectrum of juvenile xanthogranulomas is critical for proper diagnosis.
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PMID:Cutaneous xanthogranulomas, hepatosplenomegaly, anemia, and thrombocytopenia as presenting signs of juvenile myelomonocytic leukemia. 2000 Aug 79


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