Peripheral T-cell lymphoma associated with hemophagocytic syndrome

Brunangelo Falini, Stefano Pileri, Ignacio De Solas, Massimo F. Martelli, David Y. Mason, Georges Delsol, Kevin C. Gatter, Marta Fagioli

Research output: Contribution to journalArticle

Abstract

Nine patients with an acute disease characterized by high fever, loss of weight, prominent hepatosplenomegaly, slight or no lymphadenopathy, abnormal liver function tests, and profound pancytopenia are reported. In all cases, the disease presented in the absence of any pre-existing disease or immunosuppressive therapy. In seven of the nine patients, survival was very short (mean = 7 weeks). Two patients are still alive: one had a relapse 24 months after the initial diagnosis, while the other is in complete remission. The main pathological feature was the infiltration of the marrow, spleen and liver by neoplastic T cells. accompanied by an exuberant hyperplasia of benignlooking, hemophagocytizing histiocytes. The term "peripheral T-cell lymphoma with hemophagocytic syndrome" is proposed for this condition. Retrospective analysis of stored paraffin material (1949 to 1965) from the Radcliffe Infirmary files suggests that at least some of the cases designated as "histiocytic medullary reticulosis" by Scott and Robb-Smith were examples of the syndrome herein described.

Original languageEnglish
Pages (from-to)434-444
Number of pages11
JournalBlood
Volume75
Issue number2
Publication statusPublished - Jan 15 1990

Fingerprint

Peripheral T-Cell Lymphoma
Hemophagocytic Lymphohistiocytosis
T-cells
Liver
Pancytopenia
Preexisting Condition Coverage
Histiocytes
Liver Function Tests
Acute Disease
Immunosuppressive Agents
Infiltration
Paraffin
Hyperplasia
Weight Loss
Fever
Spleen
Bone Marrow
T-Lymphocytes
Recurrence
Survival

ASJC Scopus subject areas

  • Hematology

Cite this

Falini, B., Pileri, S., De Solas, I., Martelli, M. F., Mason, D. Y., Delsol, G., ... Fagioli, M. (1990). Peripheral T-cell lymphoma associated with hemophagocytic syndrome. Blood, 75(2), 434-444.

Peripheral T-cell lymphoma associated with hemophagocytic syndrome. / Falini, Brunangelo; Pileri, Stefano; De Solas, Ignacio; Martelli, Massimo F.; Mason, David Y.; Delsol, Georges; Gatter, Kevin C.; Fagioli, Marta.

In: Blood, Vol. 75, No. 2, 15.01.1990, p. 434-444.

Research output: Contribution to journalArticle

Falini, B, Pileri, S, De Solas, I, Martelli, MF, Mason, DY, Delsol, G, Gatter, KC & Fagioli, M 1990, 'Peripheral T-cell lymphoma associated with hemophagocytic syndrome', Blood, vol. 75, no. 2, pp. 434-444.
Falini B, Pileri S, De Solas I, Martelli MF, Mason DY, Delsol G et al. Peripheral T-cell lymphoma associated with hemophagocytic syndrome. Blood. 1990 Jan 15;75(2):434-444.
Falini, Brunangelo ; Pileri, Stefano ; De Solas, Ignacio ; Martelli, Massimo F. ; Mason, David Y. ; Delsol, Georges ; Gatter, Kevin C. ; Fagioli, Marta. / Peripheral T-cell lymphoma associated with hemophagocytic syndrome. In: Blood. 1990 ; Vol. 75, No. 2. pp. 434-444.
@article{46f1dd6959d44f669c1c53c728842745,
title = "Peripheral T-cell lymphoma associated with hemophagocytic syndrome",
abstract = "Nine patients with an acute disease characterized by high fever, loss of weight, prominent hepatosplenomegaly, slight or no lymphadenopathy, abnormal liver function tests, and profound pancytopenia are reported. In all cases, the disease presented in the absence of any pre-existing disease or immunosuppressive therapy. In seven of the nine patients, survival was very short (mean = 7 weeks). Two patients are still alive: one had a relapse 24 months after the initial diagnosis, while the other is in complete remission. The main pathological feature was the infiltration of the marrow, spleen and liver by neoplastic T cells. accompanied by an exuberant hyperplasia of benignlooking, hemophagocytizing histiocytes. The term {"}peripheral T-cell lymphoma with hemophagocytic syndrome{"} is proposed for this condition. Retrospective analysis of stored paraffin material (1949 to 1965) from the Radcliffe Infirmary files suggests that at least some of the cases designated as {"}histiocytic medullary reticulosis{"} by Scott and Robb-Smith were examples of the syndrome herein described.",
author = "Brunangelo Falini and Stefano Pileri and {De Solas}, Ignacio and Martelli, {Massimo F.} and Mason, {David Y.} and Georges Delsol and Gatter, {Kevin C.} and Marta Fagioli",
year = "1990",
month = "1",
day = "15",
language = "English",
volume = "75",
pages = "434--444",
journal = "Blood",
issn = "0006-4971",
publisher = "American Society of Hematology",
number = "2",

}

TY - JOUR

T1 - Peripheral T-cell lymphoma associated with hemophagocytic syndrome

AU - Falini, Brunangelo

AU - Pileri, Stefano

AU - De Solas, Ignacio

AU - Martelli, Massimo F.

AU - Mason, David Y.

AU - Delsol, Georges

AU - Gatter, Kevin C.

AU - Fagioli, Marta

PY - 1990/1/15

Y1 - 1990/1/15

N2 - Nine patients with an acute disease characterized by high fever, loss of weight, prominent hepatosplenomegaly, slight or no lymphadenopathy, abnormal liver function tests, and profound pancytopenia are reported. In all cases, the disease presented in the absence of any pre-existing disease or immunosuppressive therapy. In seven of the nine patients, survival was very short (mean = 7 weeks). Two patients are still alive: one had a relapse 24 months after the initial diagnosis, while the other is in complete remission. The main pathological feature was the infiltration of the marrow, spleen and liver by neoplastic T cells. accompanied by an exuberant hyperplasia of benignlooking, hemophagocytizing histiocytes. The term "peripheral T-cell lymphoma with hemophagocytic syndrome" is proposed for this condition. Retrospective analysis of stored paraffin material (1949 to 1965) from the Radcliffe Infirmary files suggests that at least some of the cases designated as "histiocytic medullary reticulosis" by Scott and Robb-Smith were examples of the syndrome herein described.

AB - Nine patients with an acute disease characterized by high fever, loss of weight, prominent hepatosplenomegaly, slight or no lymphadenopathy, abnormal liver function tests, and profound pancytopenia are reported. In all cases, the disease presented in the absence of any pre-existing disease or immunosuppressive therapy. In seven of the nine patients, survival was very short (mean = 7 weeks). Two patients are still alive: one had a relapse 24 months after the initial diagnosis, while the other is in complete remission. The main pathological feature was the infiltration of the marrow, spleen and liver by neoplastic T cells. accompanied by an exuberant hyperplasia of benignlooking, hemophagocytizing histiocytes. The term "peripheral T-cell lymphoma with hemophagocytic syndrome" is proposed for this condition. Retrospective analysis of stored paraffin material (1949 to 1965) from the Radcliffe Infirmary files suggests that at least some of the cases designated as "histiocytic medullary reticulosis" by Scott and Robb-Smith were examples of the syndrome herein described.

UR - http://www.scopus.com/inward/record.url?scp=0025034736&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0025034736&partnerID=8YFLogxK

M3 - Article

C2 - 2153036

AN - SCOPUS:0025034736

VL - 75

SP - 434

EP - 444

JO - Blood

JF - Blood

SN - 0006-4971

IS - 2

ER -