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At least 19 recordsLinked to original sources
PubMed1998

Malignant histiocytic disorders in children. Clinical and therapeutic approaches with a nosologic discussion.

Malignant histiocytic disorders, other than leukemias, are extremely rare in childhood. Despite unresolved nosologic and terminologic difficulties, they should be classified according to the lineage of the aberrant cells in a given tumor. There are no common and typical clinical presentations, nor are there established treatment modalities available. For the disseminated forms, aggressive systemic treatment modalities--similar if not identical to those used for large cell anaplastic lymphomas--appear to be the best treatment option. For the localized forms, which are primarily dendritic cell sarcomas, a more localized and individualized therapy is appropriate.

Child↗
PubMed1995

Malignant histiocytosis: a reassessment of cases formerly classified as histiocytic neoplasms and review of the literature.

Malignant histiocytosis (MH) and true histiocytic lymphoma (THL) are hematopoietic malignancies of the mononuclear phagocytic system distinguished from each other by clinical presentation and presumed cell of origin. THL present as a localized mass derived from the fixed tissue histiocyte which may or may not disseminate. MH originates from the circulating monocyte or tissue macrophage and is characterized by a syndrome of systemic symptoms, pancytopenia, adenopathy, hepatosplenomegaly, and wasting. The distinction between MH and THL is at times arbitrary and overlap exists between these syndromes. The clinicopathologic studies that defined these entities were performed prior to the development of immunophenotyping and other molecular techniques currently used to ensure proper classification of hematopoietic malignancies. Nine patients from the University of Minnesota originally diagnosed with MH were retrospectively analyzed using a panel of antibodies reactive against T cell, B cell, and myelomonocytic antigens. Only one patient was reclassified as a possible histiocytic malignancy after reevaluation. Similar immunophenotyping studies have also shown cases previously diagnosed as MH or THL express lymphoid antigens, and would now be classified as Ki-1 positive anaplastic large cell lymphoma (ALCL) or some other hematopoietic neoplasm. These results indicate true histiocytic neoplasms are extremely rare, and previous concepts concerning clinical presentation and therapeutic outcome of the entities are inaccurate. In this paper we summarize the results of multiple retrospective analyses of cases previously diagnosed as MH or THL, including our experience at University of Minnesota, to illustrate the overall rarity of these entities. The current literature on malignant histiocytic disorders is reviewed, and the clinical presentation of patients determined to have histiocytic malignancies using contemporary analytical techniques is discussed.

Histiocytic Disorders, Malignant↗
PubMed2003

Histiocytic lesions involving the bone marrow.

Histiocytic lesions involving the bone marrow include a number of reactive and neoplastic disorders. This article discusses the morphologic, immunophenotypic, and genotypic features of a variety of diseases associated with histiocytes and/or monocytes. Lysosomal storage disorders and hemophagocytic syndromes are often first diagnosed by bone marrow examination. Granulomas involving the bone marrow may also be the first indication of a systemic disorder. Apart from acute and chronic monocytic leukemias, the bone marrow is rarely involved by malignant histiocytic disorders, of which Langerhans cell histiocytosis is the most common.

Bone Marrow↗
PubMed2006

Aggressive histiocytic disorders that can involve the skin.

Histiocytoses are a heterogeneous group of disorders that are characterized by the proliferation and accumulation of reactive or neoplastic histiocytes. Three classes of histiocytoses have been defined: class I, Langerhans cell disease; class II, non-Langerhans cell histiocytic disease without features of malignancy; and class III, malignant histiocytic disorders. Although the disorders in classes I and II usually have a benign appearance on histology and are commonly non-aggressive and self-healing, some can cause debilitating or even fatal outcomes. Such cases beg the question: what stimulates aggressive behavior of a classically benign disease? New molecular information may now provide insight into the driving force behind many of the aggressive histiocytoses. In this article, we review Langerhans cell disease and seven aggressive histiocytoses that can involve skin, discuss histologic features that may forecast a poor prognosis, and discuss the molecular findings that help to explain the pathophysiology of these aggressive histiocytic disorders.

Histiocytosis↗
PubMed1994

Malignant histiocytosis and large cell anaplastic (Ki-1) lymphoma in childhood: guidelines for differential diagnosis--report of the Histiocyte Society.

A workshop of the Histiocyte Society was recently held, in order to discuss the problems and confusion of malignant histiocytosis (MH) and large cell anaplastic (Ki-1) lymphoma (LCAL). The aims of this workshop were to clarify the terminology for malignant histiocytic disorders and LCAL and to produce reliable criteria for diagnosis of both MH and LCAL. This article summarises the conclusions reached.

Antigens, CD↗
PubMed1981

[Applications of the peroxidase-anti-peroxidase technique in dermatology (author's transl)].

The immunohistological findings are reported in 62 cases of malignant lymphomas, pseudolymphomas and malignant histiocytic disorders of the skin. Paraffin-embedded tissue was analyzed with the Peroxidase-Antiperoxidase (PAP) Technique for the presence of intracytoplasmic immunoglobulin (IgM, IgA, IgG, Kappa, Lambda) ana lysozyme. It can be stated that the PAP technique appears to be a valuable aid in interpreting and differentiating selected material of cutaneous lymphoproliferative and histiocytic disorders. The method supplements routine histological and histochemical staining procedures.

Histiocytoma, Benign Fibrous↗
PubMed2004

Cutaneous true histiocytic malignancy: true histiocytic lymphoma.

True histiocytic malignancies (THM) are controversial disorders that are being re-evaluated with modern cellular and molecular biology techniques. True histiocytic lymphoma (THL) is a low-incidence, poor-prognosis THM. It mainly affects the skin, gastrointestinal tract, and bone tissues. Occasionally, skin lesions are the presenting clinical features. A patient with initially cutaneous THL is described. This and other reported cases confirm that THM are a nosologic entity by themselves.

Aged↗
PubMedDate not supplied

[Polyradicular neural syndrome in a patient with leukemic variant of malignant histiocytosis].

Malignant histiocytosis is rapidly progressive, fatal disorder characterised by systemic proliferation of abnormal histiocytic cells. We report a case of a 23-year old patient with leukaemia variant of malignant histiocytosis and nervous system involvement as polyradiculoneural syndrome. The leukaemia variant as well as nervous system involvement are extremely rare in malignant histiocytosis.

Adult↗
PubMed2003

Reticulohistiocytoma of the external auditory canal.

We report the first documented case of reticulohistiocytoma of the external auditory canal in a 15-year-old male. Reticulohistiocytoma is a rare diagnosis in children. Reticulohistiocytoma, a benign lesion, belongs to a family of histiocytosis disorders which range from benign to malignant. Although histiocytic disorders are uncommon, they should be included in differential diagnosis of pediatric aural polyps.

Adolescent↗
PubMed1996

Histiocyte disorders.

Histiocyte disorders are characterised by tissue infiltration with cells of monocyte/macrophage lineage, with two disorders, Langerhans' cell histiocytosis (LCH) and haemophagocytic lymphohistiocytosis (HLH) accounting for the overwhelming majority of cases in childhood and, apart from monocyte variants of acute myeloid leukaemia, histiocytic malignancy is very rare. Although both LCH and HLH are considered reactive disorders, the prognosis of these conditions differs greatly, LCH is usually self limiting, with a mortality of 10%, but HLH is usually fatal, with a mortality of over 80% in the absence of bone marrow transplantation. Increased levels of cytokines have been demonstrated in these disorders, and may be responsible for many of the clinical features: it is unclear whether histiocytes themselves, or other immune cells, particularly T lymphocytes, are the abnormal cell population. Due to the rarity of histiocyte disorders, collaborative studies are essential to improve understanding and advance treatment.

Child, Preschool↗
PubMed2006

Canine hemophagocytic histiocytic sarcoma: a proliferative disorder of CD11d+ macrophages.

Histiocytic disorders of dogs include histiocytoma, localized histiocytic sarcoma (HS), disseminated HS (malignant histocytosis), and the reactive histiocytoses: cutaneous and systemic. A common element to these diseases is proliferation of dendritic cells (DC) of either Langerhans cell (epithelial DC) or interstitial DC lineage. In this report, 17 dogs with hemophagocytic HS are described. Breeds affected included Bernese Mountain Dog (6), Golden Retriever (4), Rottweiler (3), Labrador Retriever (2), a mixed-breed dog, and a Schnauzer, which were from 2.5 to 13 years old. The dogs presented with Coombs negative responsive anemia in 16/17 dogs (94%), thrombocytopenia in 15/17 dogs (88%), hypoalbuminemia in 16/17 dogs (94%), and hypocholesterolemia in 11/16 dogs (69%). All dogs died or were euthanized. The clinical course ranged from 2 to 32 weeks (mean 7.1 weeks). Diffuse splenomegaly with ill-defined masses was consistently present. Microscopic lesions were prevalent in spleen, liver, lung, and bone marrow. Metastasis occurred by insidious intravascular invasion with minimal mass formation. Histiocytes were markedly erythrophagocytic and accompanied by foci of extramedullary hemopoiesis. Cytologically, the histiocytes varied from well differentiated to atypical, with atypia more prevalent in spleen than bone marrow. These tumors arose from splenic red pulp and bone marrow macrophages, which expressed major histocompatibility complex class II and the beta2 integrin, CD11d. They had low and/or inconsistent expression of CD1 and CD11c, which are dominantly expressed by canine nonhemophagocytic HS of DC origin. Canine histiocytic proliferative diseases now encompass proliferation of all members of the myeloid histiocytic lineage: Langerhans cells, interstitial DC, and macrophages.

Animals↗
PubMed1984

Malignant histiocytosis in the oropharynx.

Malignant histiocytosis is characterized by systemic, progressive, and invasive proliferation of malignant histiocytes. The disorder is typically accompanied by fever, general fatigue, lymphadenopathy, and hepatosplenomegaly. A case of a 21-year-old woman with primary malignant histiocytosis of the oropharynx is reported. Histologic diagnosis from the biopsy specimen was confused by infiltration of normal-appearing histiocytes and inflammatory cells. The titers of Epstein-Barr virus and the Paul-Bunnell test were elevated without atypical lymphocytes. The patient died 3.5 months after the onset of symptoms. Autopsy revealed systemic neoplastic proliferation of malignant histiocytes. A review of literature on this subject revealed no cases of malignant histiocytosis primarily involving the oropharynx.

Adult↗
PubMed1997

Prognostic factors of hemophagocytic syndrome in adults: analysis of 34 cases.

Hemophagocytic syndrome (HPS) presents with fever, pancytopenia, liver dysfunction and increase in hemophagocytic histiocytes in various organs. Although there are two major classifications of HPS in adults, malignant and reactive histiocytosis, it is often very difficult to distinguish between these disorders. We analyzed the laboratory data of patients with HPS to evaluate prognostic factors. Of 34 patients, 14 survived, and 20 died. The median age of survivors was 29.6+/-11.5 yr significantly younger than those who died (54.7+/-17.8 yr). Twenty patients had no obvious underlying disease, the other 13 had hematological malignancies or viral infections. Comparison of laboratory data revealed that nonsurvivors had significantly lower Hb and platelet values on admission. During treatment, worsening of anemia and thrombocytopenia, increase of transaminase and biliary enzymes were similarly more prominent. Risk factors associated with death were: age over 30 yr, presence of disseminated intravascular coagulation, increased ferritin and beta2-microglobulin, anemia accompanied by thrombocytopenia and jaundice. Our data suggests that patients with HPS and any of these risk factors should be treated aggressively with sufficient chemotherapy and supportive care.

Adolescent↗
PubMed1991

The histiocytoses of childhood.

The histiocytoses of childhood include Langerhans' cell histiocytosis, haemophagocytic syndrome (familial and reactive), sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease), juvenile xanthogranuloma and malignant histiocytosis. These disorders show wide variation in their clinical presentation, prognosis and genetic implications. All are characterized by localized or generalized proliferation of histiocytes, but they differ in their morphology, histochemical and immunochemical staining patterns and electronmicroscopical features. On the basis of a comprehensive clinical history and critical interpretation of morphology and immunocytochemistry using an appropriately selected panel of antibodies, a diagnosis can be reached in the majority of cases.

Child↗
PubMed2005

Histiocytic sarcoma associated with Hodgkin's disease.

Histiocytic sarcoma is a rare malignant neoplasm. It is well-known the association of Langerhans' cell histiocytosis with Hodgkin's disease but only few cases of histiocytic sarcoma associated with Hodgkin's disease was reported. We present the case of 20-years-old female patient with Hodgkin's disease with a sternal tumor mass which was diagnosed as histiocytic sarcoma. The diagnostic was established immunohistochemically, using a large battery of antibodies (S-100, CD 68, CD 34, CD 15, CD 30, Vim, NFAP) and by electron microscopy which revealed the lack of the Birbeck granules in the malignant proliferated histiocytes.

Adult↗
PubMed2002

Flow cytometric evaluation of hemophagocytic disorders in canine.

BACKGROUND: Hemophagocytic macrophages in canine bone marrow are observed in malignant histiocytosis as well as benign hemophagocytic histiocytosis. Cytomorphologic evaluation alone may be inadequate to consistently differentiate between benign and malignant forms of hemophagocytic disorders. OBJECTIVE: The purpose of this study was to evaluate the ability of flow cytometry and immunophenotyping to differentiate between benign and malignant types of hemophagocytic disorders in dogs. METHODS: Blood smears and bone marrow differential cell counts were evaluated for 10 dogs with hemophagocytic disorders. Bone marrow samples were labeled with monoclonal antibodies to CD18, MCH class-II, Thy-1, CD14, CD3, and CD21. Using flow cytometry, forward-angle versus side-angle light scatter plots were analyzed and immunophenotypes were determined. RESULTS: Scatter plots from 3 dogs with a necropsy diagnosis of malignant histiocytosis revealed 2 atypical cell clusters. One cluster contained cells of similar size or larger than immature myeloid cells and metamyelocytes. Cells in the other cluster were highly granular, with granularity similar to or greater than that of metamyelocytes. In bone marrow from dogs with malignant histiocytosis that was labeled with anti-CD14 antibody, macrophages represented 29-48% of nucleated cells. Seven dogs had a clinical or histopathologic diagnosis of benign hemophagocytic syndrome. Three of the dogs had normal cell distribution in scatter plots. Two dogs had 2 abnormal cell clusters: 1 within the immature myeloid and metamyelocyte gates and the other with granularity similar to or greater than that of metamyelocytes. The remaining 2 dogs had an atypical cell population, mostly within the immature myeloid gate. For dogs with benign hemophagocytic syndromes, 6-17% of cells in the bone marrow were CD14 positive. CONCLUSIONS: The cellular distribution in scatter plots and the total number of macrophages in bone marrow may be useful in differentiating malignant histiocytosis from benign hemophagocytic syndromes in dogs.

Animals↗