TY - JOUR
T1 - From the archives of MD Anderson Cancer Center
T2 - Aleukemic T-prolymphocytic leukemia, a rare presentation and review of the literature
AU - Nahmod, Karen A.
AU - Thakral, Beenu
AU - Aakash, F. N.U.
AU - Iyer, Swaminathan P.
AU - Medeiros, L. Jeffrey
AU - Quesada, Andres E.
N1 - Publisher Copyright:
© 2022 Elsevier Inc.
PY - 2023/2
Y1 - 2023/2
N2 - T-prolymphocytic leukemia (T-PLL) is a rare, aggressive T-cell leukemia, and patients typically present with marked peripheral blood lymphocytosis. Approximately 15–20 % of patients may present with moderate and relative stable lymphocytosis and an indolent clinical course that can persist for a few years. However, eventually these patients go on to develop marked lymphocytosis and rapidly progressive disease. We report a 72-year-old man who presented with multicompartmental lymphadenopathy and a normal complete blood count. Excision of left and right cervical lymph nodes showed replacement of the lymph node architecture by a small T-cell neoplasm positive for CD3, CD4, CD5, CD7 and TCL-1, and negative for CD8, CD20, CD30 and ALK. Subsequent bone marrow evaluation showed minimal bone marrow involvement by a T-cell neoplasm associated with TCL1A rearrangement in 11 % of cells supporting the diagnosis of T-PLL. Despite treatment, he showed progressive lymphadenopathy while remarkably maintaining normal white blood cell counts until he eventually developed leukocytosis of 110.9 × 103/uL 26 months later. Review of the literature identified only a single abstract reporting a patient with a similar lymphoma-like presentation and normal white blood cell count; however, that case showed significant bone marrow involvement in stark contrast to the current case. In summary, we report a highly unusual case of T-PLL can initially presenting with an aleukemic or lymphoma-like clinical picture, which can make establishing the diagnosis challenging.
AB - T-prolymphocytic leukemia (T-PLL) is a rare, aggressive T-cell leukemia, and patients typically present with marked peripheral blood lymphocytosis. Approximately 15–20 % of patients may present with moderate and relative stable lymphocytosis and an indolent clinical course that can persist for a few years. However, eventually these patients go on to develop marked lymphocytosis and rapidly progressive disease. We report a 72-year-old man who presented with multicompartmental lymphadenopathy and a normal complete blood count. Excision of left and right cervical lymph nodes showed replacement of the lymph node architecture by a small T-cell neoplasm positive for CD3, CD4, CD5, CD7 and TCL-1, and negative for CD8, CD20, CD30 and ALK. Subsequent bone marrow evaluation showed minimal bone marrow involvement by a T-cell neoplasm associated with TCL1A rearrangement in 11 % of cells supporting the diagnosis of T-PLL. Despite treatment, he showed progressive lymphadenopathy while remarkably maintaining normal white blood cell counts until he eventually developed leukocytosis of 110.9 × 103/uL 26 months later. Review of the literature identified only a single abstract reporting a patient with a similar lymphoma-like presentation and normal white blood cell count; however, that case showed significant bone marrow involvement in stark contrast to the current case. In summary, we report a highly unusual case of T-PLL can initially presenting with an aleukemic or lymphoma-like clinical picture, which can make establishing the diagnosis challenging.
KW - Aleukemic
KW - Differentiation state
KW - Indolent
KW - Lymph node
KW - Lymphomatous presentation
KW - T-PLL
UR - http://www.scopus.com/inward/record.url?scp=85144779907&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85144779907&partnerID=8YFLogxK
U2 - 10.1016/j.anndiagpath.2022.152077
DO - 10.1016/j.anndiagpath.2022.152077
M3 - Review article
C2 - 36549077
AN - SCOPUS:85144779907
SN - 1092-9134
VL - 62
JO - Annals of Diagnostic Pathology
JF - Annals of Diagnostic Pathology
M1 - 152077
ER -