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Results for “Histiocytosis”

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At least 19 recordsLinked to original sources

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↗

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↗

PubMed1979

Subtle clues to diagnosis by histochemistry. Histiocytosis X.

Characteristic mononuclear cells with distorted nuclei and abundant pale-staining cytoplasms together with osteoclast-like giant cells are the cytologic hallmarks of histiocytosis X. The demonstration of acid phosphatase within giant cells in paraffin-embedded sections is a valuable aid to diagnosis. The histologic differential diagnosis of histiocytosis X includes some allergic granulomas, Hodgkin's disease, myelomonocytic leukemia, mastocytosis, and malignant histiocytosis. Some technical prerequisites for accurate diagnosis of histiocytosis X are considered briefly.

Acid Phosphatase↗

PubMed1977

[Special diagnostic and therapeutic aspects of pulmonary histiocytosis X in twelve cases from 1969 to 1975 (author's transl)].

15 cases of histiocytosis X and 274 cases of histologically confirmed sarcoidosis were diagnosed during the investigation period from 1969 to 1975. Data of 12 adults with primary pulmonary histiocytosis X were evaluated in extenso. The necessary histological verification of diagnosis was only possible by open lung biopsy. Already in early stage small excavations were found by tomography in half of the cases. Eelvation of serum copper and of the index of leukocyte alkaline phosphatase was striking. In a single case antinuclear antibodies were proven. An intra patient comparison verifies corticosteroids suppressing the disease. On the occasion of a second lung biopsy in one case could be seen that after treatment no more histiocytosis-specific substrate was existing. Exacerbation and relapse during and after continuous long-term therapy were not observed. The features of histiocytosis X and sarcoidosis are set side by side in order to show differences and relations.

Adolescent↗

PubMed1977

[Unusual complications in the course of histiocytosis X (author's transl)].

In 3 patients with histiocytosis X the following complications are described: 1. A 16 year old boy suffered from generalized histiocytosis in infancy. He died after acute intracranial hypertension caused by a basilar impression. 2. Recurrent spontaneous pneumothoraces was the first symptom of an initial exclusively pulmonary histiocytosis X. 3. Exophytically growing soft tissue tumors in the late disease state of a progressive histiocytosis X.

Adolescent↗

PubMed1977

Ig surface receptors and erythrophagocytic activity of histiocytosis X cells in vitro.

Membrane receptors for Ig and IgG and receptors for immune complexes (C3) have been identified on the surface of histiocytosis X cells by direct immunofluorescence and by rosette formation. Histiocytosis X cells also avidly phagocytose human RBC and latex particles. The cells studied originated from three eosinophilic granulomas of bone and were maintained in culture for 12 days. These immunological characteristics suggest that histiocytosis X cells represent a neoplastic variant of a cellular subpopulation (Langerhans lineage) which probably belongs to the mononuclear phagocyte system.

Binding Sites↗

PubMed1979

Histiocytosis X: current concepts and a report of two cases.

The current concepts of histiocytosis X are discussed, and the literature concering this rare disease of obscure aetiology reviewed. Two case reports of histiocytosis X presenting in similar sites in young children of similar ages, but with markedly different courses, are presented. The importance of early recognition of the disease, particularly by the dental surgeon who may see the first oral signs, is stressed. Attention is drawn to the danger of misdiagnosing the oral signs of histiocytosis X, particularly as periodontal disease. The possible aetiologies are examined and current treatment regimes discussed.

Child, Preschool↗

PubMed1978

[Malignant histiocytosis (author's transl)].

According to observations in six patients and published reports, malignant histiocytosis is characterized by premature multifocal proliferation of atypical histiocytes, especially in lymph-nodes, spleen, liver, bone marrow, and lung. The diagnosis can be confirmed by electron-microscopy, and enzyme as well as immunocytochemical tests. Fever, anaemia, leukopenia (with absolute reduction in T-lymphocytes), and jaundice are frequent. Immunoglobulins are normal or polyclonally increased. Malignant histiocytosis is more like monocyte leukaemia and histiocytosis X than neoplasms of the lymphatic system.

Adult↗

PubMed1977

Histiocytosis X of the skin in an elderly man.

A diffuse truncal cutaneous eruption of six months' duration in a 76-year-old man is described. Light and electron microscopy revealed infiltration of the papillary dermis and overlying epidermis by histiocytic cells containing Langerhans granules, findings characteristic of histiocytosis X. The Langerhans cell as a probable cell of origin and unifying feature for the subtypes of histiocytosis X is discussed. It is suggested that with increased awareness of the variability of histiocytosis X, atypical presentations will be identified more frequently.

Aged↗

PubMed1977

Familial occurrence of histiocytosis.

The clinical and histological findings of four children with lethal histiocytosis are reported. The children belonged to two sibships and originated from a Swedish geographical isolate. Consanguinity between the parents was established for one of the sibships, belonging to a pedigree in which malignant disease occurred in two generations. The observations indicate that the type of histiocytosis investigated is caused by homozygosity for a single recessive gene. The association between histiocytosis and malignancy is discussed.

Consanguinity↗

PubMed1979

The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy.

Of 113 cases of sinus histiocytosis with massive lymphadenopathy, 13 patients had ophthalmic infiltrates. Eleven of the 13 had infiltrates in the orbital soft tissues, and five of these patients also had eyelid disease. One patient had infiltrates only within the eyelid, and one without disease in the orbit or eyelid had extensive infiltrates in the uveal tract. The microscopic differential diagnosis included a variety of lymphoreticular malignancies, storage diseases, histiocytosis X, rhinoscleroma, tuberculosis, and inflammatory pseudotumor of the orbit. These 13 patients with ophthalmic disease were similar clinically and pathologically to patients with sinus histiocytosis with massive lymphadenopathy who did not have ophthalmic disease.

Adolescent↗

PubMed1978

Malignant histiocytosis: a histological and ultrastructural study of lymph nodes in six cases.

A morphological, histological, and ultrastructural investigation was carried out on a series of six cases of malignant histiocytosis observed between 1973 and 1977. All lymph nodes were obtained prior to treatment. The histological findings revealed that the proliferating cell type was in all cases represented by histiocytes of varying degrees of atypia and with consistent phagocytic activity. In four cases, the histiocytic cellular proliferation also showed some degree of cohesiveness and, in two cases, there was capsular invasion. In three cases blood vessel invasion by malignant cells could be seen within the lymph node and in the surrounding tissue. On electron microscopy the tumour cells of malignant histiocytosis appeared to be pleomorphic with three types of cells: undifferentiated cells, histiocytes with variable degrees of differentiation, and cells with intermediate features. In particular, nonphagocytic and actively phagocytic histiocytes could be identified. The histological and ultrastructural data further support the idea that malignant histiocytosis is a disease that is related to the neoplastic proliferation of moderately differentiated histiocytes and their precursors. The latter may undergo various transformations with either apparently benign or definitely atypical morphological features.

Adolescent↗

PubMed1978

Sinus histiocytosis with massive lymphadenopathy (Rosai and Dorfman) and significant skin involvement.

Clinicopathological features of this female patient described here, showing unusual and extremely marked sinus histiocytosis of lymph nodes with considerable systemic lymphadenopathy, accompanying fever as well as acceleration of erythrocyte sedimentation rate fairly well coincided with the symptoms of "Sinus Histiocytosis With Massive Lymphadenopathy" (Rosai and Dorfman). The onset of the present case was in 1966 when the patient was 10 years of age, and she has been followed-up for over 10 years. While persisting to display active clinical manifestations, immunological abnormality with thymus involution was demonstrated--deterioration of cell-mediated immunity, but with non-remarkable humoral immunological data, except for hypergammaglobulinemia with elevation of IgG. In 1973 lymphadenopathy as well as skin eruption extended over the extremities and abnormalities of hematological and serological nature began to improve and at the present date the patient is uneventful. This is the first recorded case of "Sinus Histiocytosis With Massive Lymphadenopathy" (Rosai and Dorfman) in Japan. Considerable skin involvement in the upper and lower extremities was a significant feature and unique in this case.

Adult↗

PubMed1978

Malignant histiocytosis: a clinicopathologic study of 18 consecutive cases.

The clinical records and histologic material of 18 consecutive patients with malignant histiocytosis were reviewed. The age of the patients ranged from 20 months to 72 years (median 35 years). There were 14 males and 4 females (3.5:1). Lymph node and liver enlargement, fever, and skin nodules were the most common physical findings; and leukocytosis was frequently the most abnormal laboratory test. Seven of 18 patients died, and their survival ranged from 1 to 15 months (median 8 months) after histopathologic diagnosis. The histologic findings on lymph nodes, spleen, liver, bone marrow, and skin were investigated with special reference to both the cellular composition and the pattern of lymph node involvement. Vascular invasion of small perinodal vessels was observed in 4 fatal cases. The absence of capsular invasion and the lack of cohesiveness among atypical proliferating histiocytes of malignant histiocytosis appeared to be inconstant. Sequential lymph node biopsies revealed in later stages the extension of the histiocytic proliferation from the sinuses into the cords and the complete obliteration of the nodal structures. The radiologic investigations yielded numerous pathologic findings that were consistent with the dissemination of the disease. Complete response to initial treatment was achieved in patients that were treated with radiotherapy and/or chemotherapy. Complete response with chemotherapy was achieved only when the treatment included adriamycin. The histologic and clinical features of the present series provide future evidence for the recognition of malignant histiocytosis as a distinct clinical and pathologic entity.

Adolescent↗

PubMed1979

Disseminated histiocytosis and intestinal malakoplakia. Occurrence due to Mycobacterium intracellulare infection.

Disseminated histiocytic proliferation and intestinal malakoplakia due to Mycobacterium intracellulare occurred in a 2-year-old white boy. Light and electron microscopic examination of autopsy material disclosed disseminated histiocytosis and malakoplakia of the intestine with numerous intracellular acid-fast bacilli. The isolated organism produced a similar disseminated histiocytosis and finally death when injected into experimental animals. A review of the literature showed only 12 previously reported cases of disseminated histiocytosis due to M intracellulare infection. We were unable to find a previous report of malakoplakia due to nontuberculous mycobacterial infection.

Autopsy↗

PubMed1977

[Colonic histiocytosis (author's transl)].

Macrophages accumulating various substances can be detected in the mucosa of the small and large bowel under physiological and various pathological conditions. Among these the so-called PAS-positive macrophages have attracted much attention in recent times. Abundant occurrence of such cells in the intestinal mucosa has been termed "colonic histiocytosis". The occurrence of PAS-positive macrophages was investigated in 200 unselected and otherwise normal biopsy specimens of rectal mucosa; no correlation was found between the occurrence of these cells on the one hand and any intestinal or extraintestinal disease on the other. PAS-positive macrophages were mostly found close to the surface of the mucosa or to the cryptal epithelium as well as between the crypts. It is suggested to abandon the term "colonic histiocytosis" since it induces a false impression of a disease entity in the clinician (and may be related falsely e.g. to "histiocytosis X", and since the clinician may tend to attribute unnecessary importance to this harmless finding.

Adult↗

PubMed1977

The treatment of advanced histiocytosis-X with sequential hemibody irradiation.

A patient with systemic histiocytosis was treated with hemibody irradiation, vincristine, and prednisone. The patient died of an extensive peripheral neuropathy. An autopsy revealed gross histiocytosis-X involvement of the unirradiated tissues, and no evidence of disease within the field of irradiation. A treatment protocol employing sequential hemibody irradiation is discussed.

Histiocytosis, Langerhans-Cell↗

PubMed1978

Infantile histiocytosis X.

Skin biopsies and a lymphnode of three children with infantile Histiocytosis-X (Letter-Siwe Disease) were studied with enzymehistochemical and sheep-erythrocyte rosetting techniques. The majority of cells making up the infiltrates of skin and lymphnode showed rather weak acid phosphatase and nonspecific esterase activity but considerable leucyl-beta-naphtylamidase activity. Sheep-erythrocyte rosetting techniques performed on frozen sections indicated the presence of receptors for the Fc fragment of IgG, but no receptors for C3 could be demonstrated. Cells with the same enzymehistochemical characteristics could be found in thymus-dependent areas of normal spleen, of normal and reactive lymphnodes and in thymic medulla but not in B-cell areas or thymic cortex. It is suggested that Histiocytosis-X cells belong to the Mononuclear Phagocyte System and that they are related to or identical with cells normally present in the thymus dependent areas of the lymphoid tissue involved with the functioning of cell-mediated immunity.

Acid Phosphatase↗