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Titolo:
FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS ASSOCIATED WITH DISSEMINATED T-CELL LYMPHOMA - A REPORT OF 2 SIBLINGS
Autore:
MACHE CJ; SLAVE I; SCHMID C; HOEFLER G; URBAN CE; SCHWINGER W; WINTER E; HULLA W; ZENZ W; HOLTER W;
Indirizzi:
GRAZ UNIV,KINDERKLIN,AUENBRUGGERPL 30 A-8036 GRAZ AUSTRIA GRAZ UNIV,INST PATHOL A-8036 GRAZ AUSTRIA UNIV VIENNA,INST IMMUNOL A-1090 VIENNA AUSTRIA
Titolo Testata:
Annals of hematology
fascicolo: 2, volume: 69, anno: 1994,
pagine: 85 - 91
SICI:
0939-5555(1994)69:2<85:FHLAWD>2.0.ZU;2-U
Fonte:
ISI
Lingua:
ENG
Soggetto:
ACUTE LYMPHOBLASTIC-LEUKEMIA; NATURAL-KILLER ACTIVITY; ERYTHROPHAGOCYTIC LYMPHOHISTIOCYTOSIS; MALIGNANT HISTIOCYTOSIS; INTERLEUKIN-2 RECEPTOR; GENE REARRANGEMENTS; DIFFERENTIAL-DIAGNOSIS; ANTIGEN RECEPTOR; NEOPLASMS; EPIPODOPHYLLOTOXIN;
Keywords:
FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; HEMOPHAGOCYTOSIS; T CELL LYMPHOMA;
Tipo documento:
Note
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
49
Recensione:
Indirizzi per estratti:
Citazione:
C.J. Mache et al., "FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS ASSOCIATED WITH DISSEMINATED T-CELL LYMPHOMA - A REPORT OF 2 SIBLINGS", Annals of hematology, 69(2), 1994, pp. 85-91

Abstract

Two siblings with evidence of disseminated T-cell lymphoma at the time of diagnosis of familial hemophagocytic lymphohistiocytosis (FHL) are reported, an association which has not been described previously. The first child with typical clinical and laboratory features of FHL died shortly after admission, before diagnosis could be established. Retrospective analysis of autoptic tissue revealed marked hemophagocytosisas well as morphological and immunohistochemical features suggestive of disseminated T-cell lymphoma. In the second child, FHL was diagnosed in time. Subsequent histologic investigation of bone marrow biopsiesdisplayed a focal infiltration by T-cell lymphoma. DNA hybridization studies provided evidence of a monoclonal T-cell receptor beta chain gene rearrangement. Following conventional chemotherapeutic induction for FHL, the patient received an allogeneic bone marrow transplant (BMT) from a related healthy donor. Currently, 17 months after BMT, the boy is in unmaintained remission from FHL and T-cell lymphoma. The current pathogenetic concepts for FHL and a possible relationship between T-cell lymphoma and FHL are discussed.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 26/09/20 alle ore 05:47:08