An acute form of T gamma lymphoproliferative disease presenting with massive splenomegaly--importance of immunophenotyping for diagnosis.
We describe a case of T gamma lymphoproliferative disease (T gamma LPD) which presented in an uncustomary acute onset in an adult with massive splenomegaly. Morphologically the cells represented monocytic leukemia. Karyotyping and equivocol special stain results suggested hairy cell leukemia. Gene rearrangement indicated a T lymphocytic malignancy. Immunocytochemistry stains were not definitive. Immunophenotyping by flow cytometry defined the cells as consistent with T gamma LPD (CD45+, CD56+, CD2+, CD3+, CD11b+, and CD38+; some cells CD8+; and CD57-). Although the cells did not have spontaneous activity, which is often the situation for most cases of T gamma LPD, the cells could be partially induced with exogenous interleukin 2 to exhibit in vitro cytotoxicity against a natural killer lymphocyte-susceptible target cell line (K562) but not a lymphocyte-activated killer target cell line (HEPG2). This report hopefully continues to increase the awareness of T gamma LPD as well as demonstrates an unusual acute form which could have been misdiagnosed unless a multidisciplinary approach, especially including flow cytometric immunophenotyping, was used to evaluate the patient.
Warzynski
M J
MJ
West Michigan Flow Cytometry Service, Ferguson Hospital, Grand Rapids 49503.
Rosen
M H
MH
Golightly
M G
MG
Steingart
R H
RH
Otto
R N
RN
Rezuke
W N
WN
Johnson
M L
ML
D000208:Acute Disease; D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D005434:Flow Cytometry; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D016130:Immunophenotyping; D008099:Liver / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D013163:Splenomegaly / Q000209:etiology; D013601:T-Lymphocytes / Q000276:immunology