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Epstein-Barr virus (EBV)-induced CD30+ natural killer cell-type malignancy resembling malignant histiocytosis: malignant transformation in chronic active EBV infection associating hypogammaglobulinemia.

A 27-year-old male suffered from Epstein-Barr virus (EBV)-related liver dysfunction with persistent hypogammaglobulinemia. IgG titers to EBV antigens were significantly high, while other hepatitis markers were negative. Liver biopsy disclosed active intralobular inflammation. Two years later, he manifested persistent fever, leukopenia, effusions and hypoproteinemia, and his general condition worsened progressively. The peripheral blood small lymphocytes predominantly expressed natural killer (NK)-like phenotypes (CD2+, CD7+, CD16+, CD56+). Hepatosplenomegaly and marked elevation of serum lacti

True histiocytic malignancy associated with a malignant teratoma in a patient with 46XY gonadal dysgenesis.

The relatively frequent association of hematologic neoplasia and primary mediastinal germ cell tumors has been reported. Of these hematologic malignancies, nine were classified as malignant histiocytosis or acute monoblastic leukemia, and all occurred in males. We now report on a patient who was phenotypically female, with 46XY gonadal dysgenesis, and who developed a true histiocytic malignancy that presented as a large hepatic tumor and also involved the spleen, right kidney, and lymph nodes. Twenty-six months before the development of the histiocytic malignancy, an ovarian malignant teratoma

Cognitive and problem solving training in children with cancer: a pilot project.

BACKGROUND: Neurocognitive sequelae are among the most debilitating late effects experienced by survivors of childhood cancer, with far reaching consequences for educational, social, and adaptive development. Empirically validated interventions to address such disease and treatment related psychosocial morbidities are needed. PROCEDURE: We conducted a pilot study to evaluate participants' acceptance and impact of a 15-session, clinic-based training program to teach compensatory learning and problem-solving skills in survivors with cognitive deficits. The intervention program consisted of 5 co

Regulation of TNF-alpha and IL-1 gene expression during TPA-induced differentiation of "Malignant histiocytosis" DEL cell line t(5;6) (q35:p21).

The production of TNF-alpha and IL-1 alpha and beta molecules has been shown to be associated with the proliferation and activation of cells of the monocyte/macrophage series, the intermediate steps in the synthesis of these molecules have been less investigated. Unstimulated and TPA stimulated DEL cells (a CD30-positive, t(5;6)(q35;p21) malignant histiocytosis cell line) were used to study the expression of TNF-alpha and IL-1 genes and to evaluate, by nuclear run-on assay and biological measurements, the control of their transcription and the level of protein production. To refine this analys

[Extranodal Rosai-Dorfman disease of upper respiratory tract: a clinicopathologic study].

OBJECTIVE: To study the clinicopathologic features and differential diagnosis of extranodal Rosai-Dorfman disease (RDD) of the upper respiratory tract. METHODS: The clinical, pathologic and immunohistochemical features of 10 cases of RDD were evaluated. RESULTS: Among the 10 cases studied, there were 3 males and 7 females. The age of patients ranged from 20 to 61 years old (mean 38 years). The lesion arose in the nasal cavity (7 cases), nasopharynx (2 cases) or hard palate to trachea (1 case). Most of the patients presented with nasal obstruction, rhinorrhagia or tumor mass in the nasal/naso

NTP Toxicology and Carcinogenesis Studies of 1,3-Butadiene (CAS No. 106-99-0) in B6C3F1 Mice (Inhalation Studies).

1,3-Butadiene is produced in large volumes for use in the manufacture of synthetic rubber and of thermoplastic resins. In previous inhalatio … not observed. Statistically significant increases occurred in the incidences of malignant lymphoma; histiocytic sarcoma; cardiac hemangiosarcoma; harderian gland adenoma; hepatocellular adenoma and carcinoma; alveolar/bronchiolar adenoma and carcinoma; mammary gland carcinoma, adenoacanthoma, and malignant mixed tumor (females only); benign and malignant ovarian gran …

Genotype markers and proto-oncogene analysis in the CD30-positive "malignant histiocytosis" DEL cell line with t(5;6)(q35;p21).

The DEL cell line isolated from a patient who died of malignant histiocytosis exhibits a reciprocal chromosomal translocation t(5;6)(5q35;6p21). The cells were analyzed for Ig(Jh), TCR beta-gene rearrangements and proto-oncogene expression pattern, using a panel of molecularly cloned probes that included c-fms, c-myc, c-myb, c-pim, c-fos, N-myc, c-sis, c-fgr as well as the virally derived probes v-ki-ras and v-src. Consistent levels of expression of c-fms, c-myc, c-myb, c-ki-ras and c-fgr were identified in cells from several in vitro passages as well as from the heterotransplanted tumors in n

Lack of involvement of the c-fms and N-myc genes by chromosomal translocation t(2;5)(p23;q35) common to malignancies with features of so-called malignant histiocytosis.

We report the molecular, cytogenetic, and immunologic characterization of three hematologic malignancies that contained characteristic t(2;5) chromosomal translocations. The clinicopathologic features in all three cases fit the disease spectrum of so-called malignant histiocytosis (MH). All cases expressed activation antigens including Ki-1 (CD 30), but no lineage-restricted pattern of cellular antigen expression was observed. Cell lines SUP-M2 and SU-DHL-1 established from two of the cases showed rearranged beta T-cell receptor (beta TCR) genes nonproductive of full-length beta TCR mRNA and t

Childhood anaplastic large cell lymphoma Ki-1/CD30: clinicopathologic features of 19 cases.

The authors report their cumulative experience of 19 children with what was previously called malignant histiocytosis (MH) but is now considered a true lymphoma and termed anaplastic large cell lymphoma (ALCL). The median age at diagnosis was 10 years and 6 months (range 2 y, 11 m, to 15 y). There were 13 males and 6 females. Most cases presented with fever, wasting and enlarged, often tender, lymph nodes. Other features were: fleeting cutaneous rashes in 7 cases; spontaneous regression of lymph nodes and skin lesions were seen in 5 patients. Bone marrow involvement was present in 3 cases, pul

Higher in vivo protein binding of etoposide in children compared with adult cancer patients.

Etoposide is bound to plasma albumin (94%). Previous studies have revealed altered protein binding of etoposide in cancer patients. This has clinical implications since only the free fraction is considered pharmacologically active. We have studied the etoposide protein binding in 11 children (eight acute lymphocytic leukemia, two malignant histiocytosis, and one oligodendroglioma; age 1-17 years) and 46 adult patients (28 acute myelocytic leukemia, eight lymphoma, one multiple myeloma, and nine small cell lung cancer; age 38-81 years). All patients were treated with etoposide 50-200 mg/m2 i.v.

Toxicology and carcinogenesis studies of isoeugenol (CAS No. 97-54-1) in F344/N rats and B6C3F1 mice (gavage studies).

UNLABELLED: Isoeugenol is one of several structurally similar phenylpropenoid compounds produced by plants. It has been extracted from calam … ncreased in 75 and 150 mg/kg male mice. There was a significant positive trend in the incidences of histiocytic sarcoma in females, and this neoplasm occurred in multiple tissues. Incidences of respiratory metaplasia in olfactory epithelium in all exposed groups and of atrophy and hyaline droplet accumulation in all exposed groups except 75 mg/kg females were signifi …

Skin lesions in a boy with X-linked lymphoproliferative disorder: comparison of 5 SH2D1A deletion cases.

SH2D1A gene defects are the cause of X-linked lymphoproliferative disorder (XLP-1), a rare condition characterized by severe immune dysregulation. We present a patient lacking the typical symptoms of XLP-1, but experiencing a severe unusual skin condition encompassing features of dermatosclerosis and vesiculobullous skin disease. A maternal cousin of the patient was diagnosed with XLP-1 and found to carry a deletion of the SH2D1A gene. SH2D1A deletion was also identified in our patient, which offered a possible explanation for his skin symptoms. Subsequent analysis showed that the deletion in

NTP Toxicology and Carcinogenesis Studies of Phenolphthalein (CAS No. 77-09-8) in F344/N Rats and B6C3F1 Mice (Feed Studies).

Phenolphthalein is used as a laboratory reagent and acid-base indicator and in over-the-counter laxative preparations. The National Cancer I … e same between exposure groups and between males and females. Pathology Findings: The incidences of histiocytic sarcoma in 6,000 and 12,000 ppm males and females were significantly greater than those in the controls and occurred with a significant positive trend. In this study, histiocytic sarcoma was consistently observed in the liver with several other sites (e.g., …

The spectrum of giant cells in tumours of the salivary glands: an analysis of 11 cases.

In view of the different terminology for salivary gland tumours with giant cells, eleven cases were analysed by histopathology and immunocytochemistry. Four cases (three pleomorphic adenomas, one carcinosarcoma in a pleomorphic adenoma) were classified as having a foreign-body giant cell reaction, and five cases (two mucoepidermoid carcinomas, one acinic cell carcinoma, two carcinomas in pleomorphic adenomas) as having a sarcomatoid osteoclast-like giant cell reaction. In two further cases a giant cell tumour and a giant cell granuloma were associated with carcinomas in pleomorphic adenomas. A

Modulation of c-myc, c-myb, c-fos, c-sis and c-fms proto-oncogene expression and of CSF-1 transcripts and protein by phorbol diester in human malignant histiocytosis DEL cell line with 5q 35 break point.

Following exposure to phorbol ester (TPA), DEL cell line, a human malignant histiocytosis (MH) cell line, is able to differentiate along a macrophage phenotype and thus it provides a suitable model for analyzing the sequential and differential gene expression associated with monocyte/macrophage differentiation. C-myc, c-myb, c-fos, c-sis and c-fms expression were determined by Northern analysis at various times following TPA treatment. The results showed that TPA down-modulated the constitutive expression of c-myc, c-myb, and c-fms, mRNA to low but still detectable levels. Conversely, TPA-indu

Atypical cellular disorders.

Some immunologic diseases are characterized by profound loss or primary dysfunction of a given population of cells. The atypical cellular disorders discussed here all bear some similarities in that abnormal proliferations of lymphocytes and macrophages or dendritic cells result in lymphadenopathy, skin rashes, bone lesions and infiltrations of nearly any other organ system. What are the similarities and the differences between Langerhans cell histiocytosis (LCH), sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease, and Castleman's disease (CD)? Studies on LCH have

NTP Toxicology and Carcinogenesis Studies of Tetrafluoroethylene (CAS No. 116-14-3) in F344 Rats and B6C3F1 Mice (Inhalation Studies).

Tetrafluoroethylene is used in the production of polytetrafluoroethylene (Teflon(R)) and other polymers. Tetrafluoroethylene was nominated b … were considered related to tetrafluoroethylene exposure. At the end of the study, the incidences of histiocytic sarcoma (all organs) in all exposed groups of males and females were significantly greater than those in the controls and exceeded the historical control ranges for all organs. The greatest incidences of histiocytic sarcomas were observed in the liver and l …

Myeloid transformation of plasma cell myeloma: molecular evidence of clonal evolution revealed by next generation sequencing.

BACKGROUND: Plasma cell myeloma (PCM) is a neoplasm of terminally differentiated B lymphocytes with molecular heterogeneity. Although therapy-related myeloid neoplasms are common in plasma cell myeloma patients after chemotherapy, transdifferentiation of plasma cell myeloma into myeloid neoplasms has not been reported in literature. Here we report a very rare case of myeloid neoplasm transformed from plasma cell myeloma. CASE PRESENTATION: A 60-year-old man with a history of plasma cell myeloma with IGH-MAF gene rearrangement and RAS/RAF mutations developed multiple soft tissue lesions one ye

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… Zhao J Niu X Wang Z Lu H Lin X Lu Q Histiocytic sarcoma combined with acute monocytic leukemia: a case report Diagn Pathol 2015 10 110 10.1186/s13000-015-0350-9 26187047 PMC4506588 25. … Takahashi E Nakamura S Histiocytic sarcoma : an updated literature review based on the 2008 WHO classification J Clin Exp Hematop 2013 53 1 1 8 10.3960/jslrt.53.1 23801128 26. …

Tissue-Resident Myeloid and Histiocytic Cells in Health and Disease: Novel Emerging Concepts.

Although all myeloid cells are considered to derive from hematopoietic stem cells, the cells in each myeloid lineage are heterogeneous populations, and their distribution and functions vary, depending on underlying physiologic and pathologic processes, age, sex, and genetic and epigenetic signatures. In general, myeloid cells can be separated into circulating and tissue-resident cells. Tissue-resident myeloid cells can further be divided into cells derived from circulating monocytes, circulating stem cells, or local tissue-restricted stem or progenitor cells. Depending on underlying diseases a

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… P. and Qian X., “Histiocytic Sarcoma,” Archives of Pathology & Laboratory Medicine 144, no. 5 (2020): 650–654. 108. … H., Durkin M., et al., “Histiocytic Sarcoma: A Population‐Based Analysis of Incidence, Demographic Disparities, and Long‐Term Outcomes,” Blood 131, no. 2 (2018): 265–268. 110. …

Bite-size introduction to canine hematologic malignancies.

Hematologic malignancies are frequently diagnosed in dogs and result in a spectrum of clinical signs associated with specific disease types. The most frequently encountered hematologic tumors in dogs include lymphoma, lymphoid and myeloid leukemias, and mast cell, plasma cell, and histiocytic neoplasias. Coupled with the heterogeneous presentations of the different categories and subtypes of canine hematologic malignancies, outcomes for these tumors are also variable. Considering this, appropriate treatment options range from active surveillance to curative intent approaches harnessing surgica

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… Variants of canine neoplastic histiocytic disease HS, histiocytic sarcoma; iDC, interstitial dendritic cell. … Targeting MEK in a translational model of histiocytic sarcoma. Mol Cancer Ther. 2018;17(11):2439-2450. 94. Moore PF, Affolter VK, Vernau W. Canine hemophagocytic histiocytic sarcoma: a proliferative disorder of CD11d + macrophages. Vet Pathol. 2006;43(5):632-645. 95. …