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PubMed1978

Idiopathic differentiated histiocytosis.

Eighty-nine cases variously diagnosed as histiocytosis X, eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, or malignant histiocytosis from one institution were reviewed. Follow-up data were obtained for all patients. On the basis of clinicopathologic correlation, the following distinct groups were identified. 1. Disseminated histiocytosis (12 patients) with involvement of more than two organ systems at the time of recognition of the disease, affecting children less than 3 years of age (the patient's general condition is poor and hepatosplenomegaly is common. The patients do poorly, and all the patients in this group of the present study died. 2. Histiocytosis confined to bone, whether monostotic (36 patients) or polyostotic (eight patients). This type is self-healing, does not disseminate, and does not require intensive therapy. Clinically the patients are older and in good general condition during the course of the disease. Histologically histiocytes in these two groups are cytologically benign, and the whole process is inflammatory rather than neoplastic. Presence of giant cells, eosinophils, and necrosis are usually associated with better prognosis. Many neoplastic diseases and infectious processes manifest as histiocytosis. More than a third of the cases of this study diagnosed as histiocytosis proved to be other processes. This confusion contributes to differences of various reports and difficulty of interpretation of findings.

Adolescent↗
PubMed1977

Eosinophilic granuloma and its variations.

The orthopedist is most likely to encounter solitary or multiple forms of eosinophilic granuloma of bone. Management includes curettage for diagnosis and treatment with insertion of autogenous bone. Radiation therapy and chemotherapy are reserved for patients with multiple lesions and for the less common forms of histiocytosis X, i.e., Hand-Schüller-Christian disease and Letterer-Siwe disease.

Adolescent↗
PubMedDate not supplied

Electron microscopic study of the orbital lesion of Hand-Schüller-Christian disease.

The orbital bone lesion of a patient with Hand-Schüller-Christian disease was examined by electron microscopy. Vacuoles in the cytoplasm of foam cells contained granules which were shown by histochemical examination to be cholesterin. Langerhans cell granules in the histiocytes of Hand-Schüller-Christian disease showed a close resemblance to those in Langerhans cell of the epidermis, eosinophilic granuloma and Letterer-Siwe disease. Charcot-Leyden crystals appeared to be formed in the lysosomes of histiocytes in Hand-Schüller-Christian disease. From histopathological and cytological points of view, eosinophilic granuloma, Hand-Schüller-Christian disease and Letterer-Siwe disease are classified as histiocytosis X. The simultaneous appearance of Langerhans cell granules and Charcot-Leyden crystals is specific in histiocytosis X.

Bone Diseases↗
PubMed1978

[Adult Letterer-Siwe's disease. Review of literature (author's transl)].

Letterer-Siwe syndrome is usually encountered in children. It very rarely occurs in adults. 23 cases are found in the literature and analyzed: clinical signs are the same as in infantile observations. Prognosis in some cases is surprisingly very good when only the skin is involved.

Adolescent↗
PubMed1978

[2 cases of reticulohistiocytosis].

Excerpts from case histories and the results of morphological examinations of organs and tissues of a man of 17 with Letterer-Siwe disease and a man of 26 with Gaucher's disease are presented. Noteworthy is the fact of morphological similarity of eosinophilic granulema and Letterer-Siwe disease and transition of the former into the latter.

Adolescent↗
PubMedDate not supplied

A review of histiocytosis X with particular reference to eosinophilic granuloma of the lung.

Electron microscopy confirms the unity of Histiocytosis X (HX) by identifying a common marker organelle in all three forms of the disease (acute disseminated HX or Letterer-Siwe disease; chronic disseminated HX or Hand-Schuller-Christian disease; localized HX or eosinophilic granuloma). The marker organelle of HX is also found in normal Langerhans' cells: these mononuclear cells are distinct from macrophages but are similarly concerned in antigen transport. HX may therefore be considered to represent a pathological proliferation of Langerhans' cells. The natural history and the clinical, radiological and pathological features of primary pulmonary HX (eosinophilic granuloma of the lungs) are described, together with the differential diagnoses which must be considered by the histopathologist.

Adult↗
PubMed1977

Mitotic histiocytes and intranuclear Langerhans cell granules in histiocytosis X.

Several mitotic histiocytes containing Langerhans cell granules were found in the lymph nodes of Letterer-Siwe disease. Some histiocytes of Hand-Schüller-Christian disease contained Langerhans cell granules within the nuclei. These Langerhans cell granules were not in 'nuclear pseudo-inclusions,' but were freely scattered inside the nuclei. We suggest that the Langerhans cell granules may get trapped in the nucleus during mitosis, since several investigators have suggested that mitosis causes other intranuclear organelles to get trapped. We also speculate that cells containing Langerhans cell granules may divide and increase by mitosis.

Cell Nucleus↗
PubMed1992

Expression of neuron-specific enolase immunoreactivity by cutaneous and extracutaneous Langerhans-cell histiocytoses ("X").

The immunohistochemical expression of Neuron-Specific Enolase (NSE) and of S100 protein was studied in 10 cases of cutaneous and 19 cases of extracutaneous Langerhans cell histiocytoses (LCH), including acute/proliferative forms (cutaneous Letterer-Siwe disease) and chronic/granulomatous forms (eosinophilic granuloma, Hand-Schüller-Christian disease). Of the LCH cases, 18 (62%) exhibited detectable NSE-immunoreactivity as compared to 82.8% for S100. NSE expression was found more frequently and intensely within acute (as compared to chronic) forms of LCH. This result lends further support to the cellular unicity of LCH, but also suggests some degree of heterogeneity among LCH cells. It can be speculated that NSE-expression is correlated with the proliferation/activation state of (abnormal) Langerhans cells.

Bone Diseases↗
PubMed1979

Histiocytosis X of the ear and temporal bone: review of 22 cases.

Histiocytosis X encompasses three syndromes characterized by the idiopathic proliferation of histiocytes: eosinophilic granuloma, Hand-Schuller-Christian syndrome, and Letterer-Siwe disease. At the Mayo Clinic between 1926 and 1978, 22 patients with histiocytosis X had involvement of the ear or temporal bone. These patients comprised 15% of all patients with histiocytosis X seen during that period. The ages at onset of the disease ranged from 2 months to 49 years. The most frequent otologic symptom was aural discharge (15 patients), followed in frequency by swelling in the temporal region (11 patients), vertigo (6 patients), and deafness (5 patients). Clinical findings included otitis media (13 patients), otitis externa with or without granulation tissue (10 patients), and osteolytic lesions of the temporal bone (9 patients). A high index of suspicion is required to recognize the otologic manifestations of histiocytosis X for two reasons: the systemic manifestations of the disease are often so dramatic that the ear findings are overlooked, and the otologic findings of histiocytosis X can mimic more common diseases, including simple otitis externa, aural polyps, acute mastoiditis, chronic otitis media, and metastatic lesions.

Adolescent↗
PubMed1978

Histiocytosis and a leukaemoid response. A case report.

A patient with all the clinical signs of Letterer-Siwe disease is reported. The patient was unusual in that he had a severe leukaemoid reaction. To our knowledge, only 2 similar patients with reticulo-endotheliosis, histiocytic skin infiltration and leukaemoid reaction have been reported. It is possible that the disorder may represent a variant of the histiocytic proliferative disorders, characterized by both histiocytic and myeloid hyperplasia, and is thus similar to myelomonocytic leukaemia. However, it differs from the latter condition in that there is proliferation of monocytes and granulocytes in soft tissues rather than in the blood.

Blood Cell Count↗
PubMed1976

[Optimation and simplification of clinical tumor cytodiagnosis by means of cytocentrifugation].

Since the introduction of cytocentrifugation, the methods of clinical cytology have become more refined. The following advantages should be stressed: 1. speed and ease of processing, 2. highest possible cellular yield, 3. good preservation of cellular characteristics. In the light of predominantly CSF analyses in the field of pediatric oncology the above mentioned findings are clearly illustrated (ALL, AML, reticulum cell sarcomatosis, Ewing sarcoma, leptomeninx sarcoma, retinoblastoma, Letterer-Siwe's disease, familial erythrophagocytic lymphohistiocytosis, plexus papilloma). Malignancies are clearly distinguishable from benign conditions (toxic glial reactions, virus meningitides, CNS hemorrhages etc.). The higher cellular yield permits very early diagnosis of meningeal leukemia. This qualitative improvement of cytological diagnostic methods may considerably influence the choice of therapeutic procedures in the treatment of malignancies.

Centrifugation↗