Research progress on the therapy of T-cell prolymphocytic leukemia / 中国肿瘤临床

Prolymphocytic leukemia is a rare disease with typical characteristics, including aggressive progression and high mortality rate, and T-cell prolymphocytic leukemia (T-PLL) is regarded as an intractable subtype. Current therapeutic approaches mainly aim to improve their efficacy and remission against this disease. This paper presents a review about the research progress on T-PLL therapies. With remarkable research progress on leukemia pathogenesis, therapies for T-PLL have been greatly enhanced. Among treatment strategies, epigenetic therapy shows potential for clinical applications.

CD56+ T-cell Prolymphocytic Leukemia Showing a High Expression Level of TCL1 Oncogene: A Case Report with a Review of the Literature

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.

Detection of CD5 in B-cell chronic lymphoproliferative diseases by flow cytometry: a strong expression in B-cell chronic lymphocytic leukemia

PURPOSE: CD5 is a T cell marker, aberrantly express in B cell chronic lymphocytic leukemia (B-CLL) and mantle cell lymphoma (MCL). Other chronic B cell malignancies including hairy cell leukemia (HCL) and B cell prolymphocytic leukemia (B-PLL) are CD5 negative or express this antigen in a weak way. In this study, CD5 expression was investigated in leukemic cells from 42 patients with chronic B cell lymphoproliferative disease. METHODS: We studied the CD5 expression in leukemic cells from 42 patients with chronic B-cell malignancies by flow cytometry. Demographic features such as age, sex and clinical date were also analyzed. RESULTS: There were 22 males and 20 females. The immunophenotyping showed that 35 cases were B-CLL, 3 B-PLL and HCL and one patient was MCL. CD5 expression was present in all B-CLL and MCL. Low expression of CD5 was observed in one patient with B-PLL and negative in all cases of HCL. CONCLUSION: Our date demonstrated that CD5 expression can help distinguish among B-CLL from HCL and B-PLL, but is similar expressed in MCL.

OBJETIVOS: CD5 é um marcador normalmente expresso nas células T e de forma aberrante nas células B da leucemia linfocítica crônica (LLC) e no linfoma de células do manto (LCM). Outras doenças linfoproliferativas crônicas como a hairy cell leukemia (HCL) e leukemia prolinfocítica de células B (LPL-B), são geralmente CD5 negativas ou expressam fracamente este antígeno. Neste trabalho investigou-se o padrão de expressão do CD5 em 42 pacientes com doenças linfoproliferativas crônicas de células B (DLC-B). METODOS: Investigamos a expressão de CD5 em células leucêmicas de 42 pacientes com DLC-B através da citometria de fluxo. Dados demográficos, tais como idade e sexo, bem como dados clínicos e laboratoriais também foram analisados. RESULTADOS: A imunofenotipagem mostrou que 35 casos foram LLC, 3 LPL-B, 3 HCL e um caso de LMC. O CD5 mostrou-se fortemente expresso em todos os casos de LLC e LMC. Baixa expressão desse antígeno foi observada em um caso de LPL-B, mostrando-se negativamente expresso em todos os casos de HCL. CONCLUSÃO: Nossos resultados demonstram que o padrão de expressão do CD5 pode auxiliar na distinção entre LLC da HCL e LPL-B, sendo no entanto similares na HCL e LCM.

A Case of Small Cell Variant of T-Cell Prolymphocytic Leukemia

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.

A Case of B-Prolymphocytic Leukemia and Another Case of B-Chronic Lymphocytic Leukemia/Prolymphocytic Leukemia / 대한혈액학회지

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-cell Prolymphocytic Leukemia Involving Entire Female Genital Tract: A case report

Prolymphocytic leukemia is a chronic lymphoproliferative disorder, characterized by prominent splenomegaly, prolymphocytes accounting for more than 55% of circulating lymphocytes, no significant peripheral lymphadenopathy and short term survival with terminal fatal multi-organ failure. We report a case of B-cell prolymphocytic leukemia in a 57-year-old woman who presented with easy bruising and arthritis for 1 year and low abdominal pain for 2 months. Physical examination revealed gingival hypertrophy and mild splenomegaly. On peripheral blood smears the leukocytes were markedly increased in number due to leukemic cells that count about 62% of leukocytes. The bone marrow aspiration smear and biopsy revealed diffuse infiltration of medium to large prolymphocytes having moderate amount of basophilic cytoplasm, round to oval nuclei with coarse chromatin, and prominent nucleoli. Abdominal pain aggravated despite chemotherapy, and pelvic computed tomography (CT) revealed a huge lobular pelvic mass which had increased in size on the follow-up CT. Total hysterectomy with bilateral adnexectomy was performed. Microscopic findings included massive infiltration of prolymphocytic cells in the uterus, upper vaginal wall, bilateral ovaries, and bilateral mesosalpinges. On immunohistochemistry, the leukemic cells showed B cell gamma light chain phenotype.

A Case of B-Prolymphocytic Leukemia / 대한혈액학회지

Prolymphocytic leukemia (PLL) is a member of chronic lymphoproliferative disorders with relatively distinct clinical, morphologic, immunologic and prognostic features. The diagnosis of PLL is determined by more than 55% of prolymphocytes in the peripheral blood. It is characterized by leukocytosis, massive splenomegaly with little or no lymphadenopathy, and male prevalence. In immunophenotyping, the majority (80%) of the cases express B cell markers and the rest (20%), T cell type. We experienced a case of B-PLL. The patient was a 65 year-old man who presented with marked leukocytosis (110.5x109/L) and 78% of characteristic prolymphocytes in the peripheral blood. The bone marrow aspirate showed 13.6% of prolymphocytes and coarse granular positivity of prolymphocytes in PAS stain. The immunophenotyping of the leukemic prolymphocytes revealed the positivity of surface immunoglobulin (IgM, lambda type), HLA- DR and CD19. The monoclonal gammopathy (IgM, lambda type) was also detectable in the patient's serum.