To improve the recognition of angioimmunoblastic T-cell lymphoma (AITL) and to reduce misdiagnosis, a case diagnosed as AITL with large granular lymphocytosis was reported and the related articles were reviewed. A series of clinical tests, pathologic examination and immunohistochemical test, TCR gene rearrangement detection by multiple PCR and assay of lymphocyte immunophenotypes were carried out. The results indicated that the patient was characterized by fever, skin rash, generalized lymphadenopathy, splenomegaly, pleural effusion, ascites, anemia and thrombocytopenia, increase of circulating large granular lymphocytes with CD3(-) and CD16(+), CD56(+) were detected, T-cell receptor γ-chain gene was rearranged. More large granular lymphocytes with abnormal mitosis were found in ascites. The histological and immunohistochemical changes observed by the lymph node biopsy were compatible with AITL, some cells of which were CD56-positive. In conclusion, AITL is characterized by aggressive progress and generally occurs in elderly patients, its clinical prognosis is poor, the large granular lymphocytosis may be an autoimmune response to the tumor cells or originate from tumor stem/progenitor cells.
Humans ; Immunoblastic Lymphadenopathy ; complications ; immunology ; pathology ; Immunophenotyping ; Leukemia, Large Granular Lymphocytic ; complications ; immunology ; pathology ; Male ; Middle Aged

Histiocytosis X(Langerhans cell histiocytosis) is a rare prolifertive disorder of Langerhans cells that includes Lettere-Siwe disease, Hand-Shiiller-Christian his ase and eosinophilic granuloma. Since many authors have reported in anintermediate and poarl classified form histiocytosis X has a namenclatural had nosologic problem. We report a case benign cutaneous variant of histiocytisis X. A four-month-old boy had shown multiple skin-colour papules on the trunk, head and nik for one month. Extensive in- vestigations failed to detcct any systemic involvement. The clixron microscopic findings of the skin biopsy specimen were Qefinitely diagnostic for histiocytosi. X Since he did not sbow evidence of internal organ involvernent and any further progression of he skin lesion, no therapy was given. Over the next two months the disease underwent spontane us and complete remission.
Biopsy ; Eosinophilic Granuloma ; Head ; Histiocytosis* ; Histiocytosis, Langerhans-Cell* ; Humans ; Langerhans Cells ; Male ; Remission, Spontaneous* ; Skin

No abstract available.
Niemann-Pick Disease, Type A*

No abstract available.
Niemann-Pick Disease, Type A*

Histiocytosis-X has several mode of presentation, from the mildest solitary eosinophilic granuloma of bone to the most severe, with multiple granulomata in bones and soft tissues. This disease previously described as eosinophilic granuloma, Hand-Schuller-Christian disease and Letterer-Siwe disease. Lichtenstein (1953) used the term histiocytosis-x to encompass the above three names. We experienced a case of histiocytosis-x which had occured at multiple site of bones. The patient has good general condition with complete subsidance of specific symtoms and signs in our thirty-eight months follow up treatment.
Eosinophilic Granuloma ; Follow-Up Studies ; Histiocytosis, Langerhans-Cell ; Humans

Eosinophilic granuloma, Hand-Schuller-Christian and Abt-Letterer-Siewe syndrome are known to be the same disease entity involving the reticulohistiocytic system of the body, although their clinical manifestations are different. So they were included under the term of Histiocytosis X and this concept has been generally accepted. The authors have experienced one case of Hand-Schuller-Christian disease and that of eosinophilic granuloma. The former was a 23 years old man with complaints of exophthalmos, polyuria and a tender palpable mass on the right parietal area and in the latter a soft pulastile mase located on the left parietal area in a 3 year old boy. There was no neurological deficits on their admission. We managed them with radiation and steroid therapy following surgical curettage.
Child, Preschool ; Curettage ; Eosinophilic Granuloma ; Exophthalmos ; Histiocytosis* ; Histiocytosis, Langerhans-Cell* ; Humans ; Male ; Polyuria ; Rabeprazole ; Young Adult

Langerhans cell histiocytosis (LCH) involves disorders previously referred as "histiocytosis X" (including eosinophilic granuloma, Letterer-Siwe, and Hand-Schuller syndrome). Its clinical patterns are various and it is a basically benign tumoral condition but with a strong tendency toward dissemination and destruction. Its natural history is unpredictable. But, in solitary bone lesion, wide resection with tumor-free margin is required in order to provide the best chance for a cure. In the majority of patients LCH is a osteolytic lesion with a predilection for calvarium and is rarely seen in the skull base and the femur. LCH of rib, especially if solitary, is relatively rare. We report two rare cases of solitary LCH developed in the rib, which were successfully treated by surgical resection.
Eosinophilic Granuloma ; Femur ; Histiocytosis ; Histiocytosis, Langerhans-Cell* ; Humans ; Natural History ; Ribs* ; Skull ; Skull Base

Eosinophilic granuloma of bone is a self-limited condition, characteristically a disease of children and young adults. It is the most benign variant of histiocytosis X. It is most common in skull, but any bone may be affected. Vertebral involvement occurs in about 10 to 15%. This benign bone destructive lesion is characterized by the presence of Langerhans cells contain Birbeck granules, infilitration of histiocytes and eosinophilic leukocytes unknown origin. We report a eosinophilic granuloma in lumbar spine of old patient.
Child ; Eosinophilic Granuloma* ; Eosinophils* ; Histiocytes ; Histiocytosis, Langerhans-Cell ; Humans ; Langerhans Cells ; Leukocytes ; Skull ; Spine* ; Young Adult

Histiocytosis is a relatively rare disorder of the reticuloendothelial system involving the proliferation of histicoytes, granulation tissue, and inflammatory cells in many different organ systems1). Thus, the three manifestations of the same basic pathologic process:Eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease have been classified as localized, chronic disseminated and acute disseminated histiocytosis-X. They were therefore included under the term histiocytosis-X and this concept has been generally accepted. The authors have experienced one case of histiocytosis-X, a rare disease. A 11 month-old femal patient presented with gradually enlarged palpable mass on the occipital area. The occipital skull was defected in a punched out fashion. The mass was completely removed. The pathologic findings revealed Histiocytosis-X and the patient was given chemotherapy.
Drug Therapy ; Eosinophilic Granuloma ; Granulation Tissue ; Granuloma ; Histiocytosis ; Histiocytosis, Langerhans-Cell* ; Humans ; Infant ; Mononuclear Phagocyte System ; Rare Diseases ; Scalp* ; Skull

No abstract available.
Leukemia, Large Granular Lymphocytic*