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 language | English |
|---|---|
| Pages (from-to) | 434-444 |
| Number of pages | 11 |
| Journal | Blood |
| Volume | 75 |
| Issue number | 2 |
| Publication status | Published - Jan 15 1990 |
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ASJC Scopus subject areas
- Hematology
Cite this
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 journal › Article
}
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.
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M3 - Article
C2 - 2153036
AN - SCOPUS:0025034736
VL - 75
SP - 434
EP - 444
JO - Blood
JF - Blood
SN - 0006-4971
IS - 2
ER -