No abstract available.
Leukemia, Prolymphocytic, T-Cell* ; T-Lymphocytes*

T-cell prolymphocytic leukemia (T-PLL) is a post-thymic T-cell malignancy that has an aggressive clinical course and it is a distinct clinico-biological entity from other T-cell disorders. It is now apparent that this disease represents a separate entity from CLL. Clinically, T-PLL presents with hepatosplenomegaly, lymphadenopathy, skin lesion, and marked lymphocytosis exceeding 100x109/L. Because its clinical course is aggressive, the treatment is difficult. We report a case of small cell variant of T-cell with a review of literatures.
Leukemia, Prolymphocytic, T-Cell* ; Lymphatic Diseases ; Lymphocytosis ; Skin ; T-Lymphocytes*

De novo B-cell prolymphocytic leukemia (B-PLL) is a distinct clinicopathologic entity that was first described in 1974 by Galton et al. B-PLL is characterized by marked lymphocytosis with predominance of prolymphocytes, often massive splenomegaly, minimal lymphadenopathy, often aggressive clinical course and frequently poor prognosis.We experienced a case of B-PLL associated with false-positive direct antiglobulin test. The patient was 52 year-old man who presented with marked leukocytosis (160.2x103/(mu)L) and 60% of characteristic prolymphocytes in the peripheral blood. The bone marrow aspirate showed dry tap and the PAS stain of peripheral blood smear showed positivity of prolymphocytes. The immunophenotyping of the leukemic prolymphocytes revealed the positivity of surface immunoglobulin (IgM, lambda type), HLA-DR, CD19 and CD5.
Bone Marrow ; Coombs Test* ; HLA-DR Antigens ; Humans ; Immunoglobulins ; Immunophenotyping ; Leukemia ; Leukemia, Prolymphocytic* ; Leukemia, Prolymphocytic, B-Cell ; Leukocytosis ; Lymphatic Diseases ; Lymphocytosis ; Middle Aged ; Splenomegaly

Alemtuzumab ; Antibodies, Monoclonal, Humanized ; Hematopoietic Stem Cell Transplantation ; Humans ; Leukemia, Prolymphocytic, T-Cell ; Transplantation, Autologous

Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Leukemia, Prolymphocytic, B-Cell ; diagnosis ; Male ; Middle Aged ; Retrospective Studies

Prolymphocytic leukemia (PL) is usually derived from B cells and shares some features with chronic lymphocytic leukemia (CLL), but it is clearly a distinct entity and defined to have more than 55% prolymphocytes. Chronic lymphocytic leukemia/prolymphocytic leukemia (CLL/PL) is a mixed type of CLL and is defined to have 11~55% prolymphocytes with intermediate features between CLL and PL. We experienced two different cases of leukemia PL and CLL/PL. On physical examination, PL (stage II, B) patient showed multiple cervical lymph node enlargement and 5cm sized splenomegaly and hepatomegaly. But CLL/PL (stage III, C) patient showed 10cm sized splenomegaly and no lymph node enlargement. On immunological phenotyping, surface markers showed 72% CD5 (+), 85% CD19 (+), and 40% SmIg (+) in PL patient and 3% CD5 (+), 90% CD19 (+) and SmIg (-) in CLL/PL patient. PL patient was refractory to chlorambucil and prednisolone chemotherapy and showed poor prognosis. CLL/PL patient did not show remarkable response to chlorambucil and prednisolone therapy.
B-Lymphocytes ; Chlorambucil ; Drug Therapy ; Hepatomegaly ; Humans ; Leukemia* ; Leukemia, Lymphocytic, Chronic, B-Cell ; Leukemia, Prolymphocytic ; Lymph Nodes ; Physical Examination ; Prednisolone ; Prognosis ; Splenomegaly

T-cell prolymphocytic leukemia (T-PLL) is a rare but highly aggressive and malignant mature T-lymphoid tumor. The diagnosis of T-PLL mainly depend on genetic characteristics, clinical manifestations, cell morphology and immunophenotype. At present, clinical treatment is mainly aimed at improving the response rate and prolonging the remission period. With the development of new molecular biology technologies, researchers have gained a deeper understanding of the pathogenesis and related genetics of T-PLL, targeted drugs, including HDAC inhibitors, JAK/STAT inhibitors, AKT inhibitors and BCL-2 inhibitors, are also under evolution and providing the new opportunities to improve the efficacy of therapy. In this review, the advances in genetics and treatment of T-PLL were summarized briefly.
Antineoplastic Agents ; Humans ; Immunophenotyping ; Leukemia, Prolymphocytic, T-Cell/genetics* ; Protein Kinase Inhibitors

OBJECTIVE: To explore and summarize the clinical characteristics and treatment of aggressive NK-cell leukemia (ANKL), and provide new insights for clinical diagnosis and treatment of this disease. METHODS: The clinical data of 7 patients with ANKL admitted to the First Affiliated Hospital of Wannan Medical College from March 2014 to July 2021 were retrospectively analyzed, and their clinical characteristics, laboratory and imaging results, treatment and outcomes were analyzed. RESULTS: Among the 7 patients, 5 were males and 2 were females, with a median age of 47 (33-69) years old. The morphology of bone marrow cells in 7 patients showed similar large granular lymphocytes. Immunophenotyping revealed abnormal NK cells in 5 cases. By the end of follow-up, 6 cases died and 1 case survived, with a median survival time of 76.9 (4-347) days. CONCLUSION ANKL is a rare disease with short course and poor prognosis. If combined with hemophagocytic syndrome (HPS), the prognosis is even worse. There is no unified treatment method at present, and the use of PD-1 inhibitors may prolong the survival in some patients.
Male ; Female ; Humans ; Middle Aged ; Aged ; Retrospective Studies ; Leukemia, Large Granular Lymphocytic ; Leukemia, Prolymphocytic, T-Cell ; Prognosis ; Lymphohistiocytosis, Hemophagocytic

T-cell prolymphocytic leukemia (T-PLL) is a rare mature post-thymic T-cell malignancy with infiltration to the blood, bone marrow, lymph node, liver, spleen and skin; this disease has a poor prognosis and an aggressive clinical course. We report here on a case of CD56+ T-PLL that was diagnosed by hematological examination, immunophenotyping and molecular studies including determining the TCL1 expression by using reverse-transcriptase polymerase chain reaction (RT-PCR), and direct sequencing of the RT-PCR product.
Bone Marrow ; Immunophenotyping ; Leukemia, Prolymphocytic, T-Cell* ; Liver ; Lymph Nodes ; Oncogenes* ; Polymerase Chain Reaction ; Prognosis ; Skin ; Spleen ; T-Lymphocytes*

<b>OBJECTIVEb>To investigate the clinical and laboratory characteristics and survival of Chinese patients with T- cell prolymphocytic leukemia (T-PLL).

<b>METHODSb>Eleven patients with T-PLL admitted in our hospital from Jan 2006 to Oct 2012 were retrospectively analyzed.

<b>RESULTSb>Of the 11 patients, nine were males and two females, with the median age of 56.0(19-69) years old. All the patients, except for three, presented with leukocytosis. The incidence of hyperleukocytosis (1/11) was less frequent than that in the British series (75%) (P=0.000). Lymphocyte counts in peripheral blood were increased in 9 of the 11 patients with the median absolute lymphocyte count (ALC) of 17.22(0.58-148.83)×10⁹/L. Superficial lymphadenopathy and splenomegaly were the most common physical signs. It was common that serum lactate dehydrogenase (LDH) and beta 2 microglobulin(β2-MG)were higher than normal level. All cases were positive for CD2/CD3/CD5/TCRαβ, negative for CD1a /HLA-DR and TdT, and most of them were strong positive for CD7 expression. By chromosome analyses, most cases. (9/10) have normal chromosome. This rate is significantly higher than that of the British and American series (3% and 25%, respectively) (P=0.000, P=0.001). The 14q11 abnormality and trisomy 8q, which are common among Western cases, were not observed in any of our cases. With a median follow-up of 23.0 months, three patients died. Two year progress free survival (PFS) and overall survival (OS) were 53.3% and 50%, respectively. There were 3 patients with PFS over a number of years, whether it should be considered as the T-chronic lymphocytic leukemia (T-CLL) is worthy of further studies.

<b>CONCLUSIONb>The common clinical manifestations of T-PLL patients were increased lymphocyte counts and lymphadenopathy as well as splenomegaly. And most cases have high level of blood LDH and β2- MG and normal chromosome karyotype.

Adult ; Aged ; Bone Marrow Examination ; China ; Female ; Humans ; Leukemia, Prolymphocytic, T-Cell ; diagnosis ; Male ; Middle Aged ; Retrospective Studies ; Young Adult