Research Advances of Genetics, Diagnosis and Treatment of T-cell Prolymphocytic Leukemia--Review / 中国实验血液学杂志

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.

Small cell variant of T-cell prolymphocytic leukemia with atypical presentation

No abstract available.

Leucémie lymphoïde chronique au Niger: une étude de 99 cas au service d'onco-hématologie de l'hôpital national de Niamey

Introduction. Le but de l'étude est de décrire les caractéristiques épidémiologiques et cliniques de la leucémie lymphoïde chronique (LLC) au Niger. C'est la première étude nigérienne spécifiquement consacrée à cette maladie. Méthodologie. Nous avons mené une étude rétrospective couvrant la période de janvier 2000 à décembre 2011 (12 ans) dans le service d'Onco-Hématologie de l'HNN. Le diagnostic de LLC était retenu sur la base d'une hyper lymphocytose sanguine > 15 000/mmᶾ associée à une infiltration médullaire de plus de 40% de lymphocytes mâtures. Les données ont été recueillies dans les dossiers de malades. Nos variables d'étude étaient les aspects épidémiologiques, cliniques et évolutifs de la maladie. Résultats. Au cours de la période d'étude, 99 patients ont été colligés soit une fréquence d'environ huit cas par an. Le sex ratio était de 0,47 et la moyenne d'âge des patients de 53,25 ans (extrêmes: 30 à 82 ans). L'échantillon était constitué de 89 % de paysans (cultivateurs, éleveurs femmes au foyer). La durée moyenne des troubles avant la première consultation était de 24 mois. Les principaux motifs de consultation étaient: la splénomégalie (81,8%), les adénopathies (38,4%) et l'anémie (21,2%). Les principaux signes physiques étaient: les adénopathies (84,8%); la splénomégalie (80,8%); la pâleur cutanéo-muqueuse (31,3%); la fièvre (29,3%) et l'hépatomégalie (25,3%). Selon la classification anatomo-clinique de Binet, 39 patients (39,4%) étaient au stade A, 16 cas (16,2%) au stade B et 44(44,4%) au stade C. Conclusion. À Niamey, la LLC est une maladie de l'adulte jeune diagnostiquée souvent à un stade avancé du fait du retard de la première consultation

Leucemia prolinfocítica T variante de células cerebriformes: presentación de un caso y revisión de la literatura / Cerebriform variant type of T cell prolymphocytic leukemia: report of one case

T cell Prolymphocytic Leukemia (T-PLL) is a rare and aggressive mature T cell Lymphocyte Leukemia. Twenty five percent of cases present as a small cell variant, and only 5% as a cerebriform variant. We report a 58 year-old man with rapidly progressive severe leukocytosis, skin lesions, lymphadenopathy, hepatosplenomegaly and pleural effusion. The lymphocytes had a cerebriform type. The diagnosis of T-PLL variant was made by morphology and immunophenotype study of peripheral blood. Karyotype was found to be complex. He was refractory to chemotherapy and died two months later.

Clinical and laboratory features of T-cell prolymphocytic leukemia in China / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical and laboratory characteristics and survival of Chinese patients with T- cell prolymphocytic leukemia (T-PLL).</p><p><b>METHODS</b>Eleven patients with T-PLL admitted in our hospital from Jan 2006 to Oct 2012 were retrospectively analyzed.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSION</b>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.</p>

Selective embolization of the internal iliac arteries for the treatment of intractable hemorrhage in children with malignancies / 소아과

PURPOSE: Acute internal hemorrhage is an occasionally life-threatening complication in pediatric cancer patients. Many therapeutic approaches have been used to control bleeding with various degrees of success. In this study, we evaluated the efficacy of selective internal iliac artery embolization for controlling acute intractable bleeding in children with malignancies. METHODS: We retrospectively evaluated the cases of 6 children with various malignancies (acute lymphoblastic leukemia, acute myelogenous leukemia, chronic myelogenous leukemia, T-cell prolymphocytic leukemia, Langerhans cell histiocytosis, and rhabdomyosarcoma), who had undergone selective arterial embolization (SAE) of the internal iliac artery at the Chonnam National University Hwasun Hospital between January 2004 and December 2009. SAE was performed by an interventional radiologist using Gelfoam(R) and/or Tornado(R) coils. RESULTS: The patients were 5 boys and 1 girl with median age of 6.9 years (range, 0.7-14.8 years) at the time of SAE. SAE was performed once in 4 patients and twice in 2, and the procedure was unilateral in 2 and bilateral in 4. The causes of hemorrhage were as follows: hemorrhagic cystitis (HC) in 3 patients, procedure-related internal iliac artery injuries in 2 patients, and tumor rupture in 1 patient. Initial attempt at conservative management was unsuccessful. Of the 6 patients, 5 (83.3%) showed improvement after SAE without complications. CONCLUSION: SAE may be a safe and effective procedure for controlling acute intractable hemorrhage in pediatric malignancy patients. This procedure may obviate the need for surgery, which carries an attendant risk of morbidity and mortality in cancer patients with critical conditions.

Immunophenotyping of mature T/NK cell neoplasm presenting as leukemia.

Introduction : Mature T/NK cell lymphomas (MTNKL) presenting as leukemia are rare and show considerable overlapping of clinical, morphological and immunophenotypic features. AIM: Critical analysis of the morphology and immunophenotypic profile of MTNKL. Materials and Methods : We reviewed 380 consecutive cases of mature lymphoid neoplasm that presented as leukemia and were diagnosed on morphology and immunophenotyping of bone marrow and/or peripheral blood samples. Results : Peripheral blood and bone marrow involvement was seen in all cases. MTNKL constituted 4% (nine cases) of all mature lymphoid neoplasms presenting as leukemia. It included four cases of T-large granular leukemia (T-LGL), two of T-cell prolymphocytic leukemia small cell variant (T-PLL), two of adult T-cell leukemia/lymphoma (ATLL) and one of primary cutaneous gamma delta T-cell lymphoma (PCGDTCL). T-LGL revealed CD4-/CD8+ phenotype in three, and CD4+/CD8+ phenotype in one case. CD56 was absent in all the cases of T-LGL. One case of T- PLL small cell variant showed CD4+/CD8- phenotype, while the other revealed CD4-/CD8+ phenotype. Both cases of ATLL showed CD4+/CD8+/CD25+ phenotype. The single case of PCGDTCL showed CD4-/CD8- phenotype pattern. CD3 and CD5 were expressed in all MTNKL. CD7 was absent in three cases of T-LGL. TCRα/β was performed in three cases of T-LGL and was positive in all. TCRα/β was also seen in both the cases of T-PLL small variant. However, TCRα/β was seen in the single case of PCGDTCL. Conclusion : Mature nodal T/NK cell neoplasms are rare and MTNKL presenting as leukemia are even rarer. There is an overlap between the immunophenotypic profiles of different MTNKL subtypes and elaborate T/NK cell panels are required for their evaluation.

Cytologic Features of Ascitic Fluid Complicated by Small Cell Variant T-cell Prolymphocytic Leukemia: A Case Report / 대한세포병리학회지

T-cell prolymphocytic leukemia (T-PLL) is a rare, mature T-cell lymphoproliferative disorder with a post-thymic mature T-cell phenotype. The disease is characterized by rapidly rising lymphocytosis, lymphadenopathy, and splenomegaly. The clinical course is usually aggressive and progresses with frequent skin lesions and serous effusions. In 25% of cases, leukemic cells are small and tumor cells may not have a discrete nucleolus under light microscopy. Although the presence of characteristic cytoplasmic protrusions or blebs in tumor cells is a common morphologic finding in the peripheral blood film irrespective of the nuclear features, small cell variants lacking the typical nuclear features can cause diagnostic problems in clinical cytology. Furthermore, the small leukemic cells can share some cytologic findings with lymphocyte-rich serous effusions caused by non-neoplastic reactive lymphocytosis as well as other small lymphocytic lymphoproliferative disorders. Here, we describe the cytological findings of ascitic fluid complicated by small cell variant T-PLL in a 54-year-old man, the cytology of which was initially interpreted as small lymphocytic malignancy such as small lymphocytic lymphoma/chronic lymphocytic leukemia.

A Case of Unspecified Mature T-cell Leukemia with Clover-shaped, Multi-lobated Nuclei / 대한혈액학회지

Mature T-cell leukemias are a group of neoplasms derived from mature or post-thymic T-cells, and a number of distinctive disease entities have been defined in the World Health Organization (WHO) classification. Here we report a 54-year-old female patient with multi-lobated atypical cells expressing the classic T-cell antigens involving multiple lymph nodes, peripheral blood, and bone marrow. The clinical, laboratory, and pathologic features of her disease did not fit into any of the entities in the WHO Classification. There was no evidence of rapidly rising lymphocyte counts, TCL1 expression, eosinophilia, erythroderma, Sezary cells, autoimmune phenomena, cytotoxic granules, nor evidence of HTLV-1 infection, and thus, T-cell prolymphocytic leukemia, Sezary syndrome, T-cell granular lymphocytic leukemia, and adult T-cell leukemia/lymphoma were all ruled out. This case suggests that further characterization and definition of the "unclassifiable" cases of mature T-cell neoplasm is needed to better understand the group of disorders.

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

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.

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

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.

Doenças linfoproliferativas T periféricas: estudo citomorfológico, imunofenotípico e sorológico / Peripheral T lymphoproliferative disorders: study cytomorphological, immunophenotypic and serological

As doenças linfoproliferativas T representam um grupo heterogêneo de noplasias linfóides que exibem um espectro muito variado de características clínicas, cito e histopatológicas...A necessidade de rastreamento epidemiológico entre doadores de sangue e população de risco (hemofílicos, prostitutas e usuários de drogas) é vital para impedir a disseminação do vírus proporcionando um controle epidemiológico eficaz