関: chronic lymphocytic leukemia、chronic T-cell leukemia、T-cell prolymphocytic leukaemia、T-PLL

WordNet

small room in which a monk or nun lives (同)cubicle
a device that delivers an electric current as the result of a chemical reaction (同)electric cell
a room where a prisoner is kept (同)jail cell, prison cell
(biology) the basic structural and functional unit of all organisms; they may exist as independent units of life (as in monads) or may form colonies or tissues as in higher plants and animals
any small compartment; "the cells of a honeycomb"
a small unit serving as part of or as the nucleus of a larger political movement (同)cadre
malignant neoplasm of blood-forming tissues; characterized by abnormal proliferation of leukocytes; one of the four major types of cancer (同)leukaemia, leucaemia, cancer of the blood
the 20th letter of the Roman alphabet (同)t
a tough youth of 1950s and 1960s wearing Edwardian style clothes (同)Teddy boy

PrepTutorEJDIC

(刑務所の)『独房』;(修道院の)小さい独居室 / (ミツバチの)みつ房,巣穴 / 小さい部屋 / 『細胞』 / 電池 / 花粉室 / (共産党などの)細胞
白血病
tritiumの化学記号

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/01/11 21:59:42」(JST)

wiki en

T-cell-prolymphocytic leukemia (T-PLL) is a mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin involvement.^[1] T-PLL is a very rare leukemia, primarily affecting adults over the age of 30. It represents 2% of all small lymphocytic leukemias in adults.^[2] Other names include T-cell chronic lymphocytic leukemia, "knobby" type of T-cell leukemia, and T-prolymphocytic leukemia/T-cell lymphocytic leukemia.^[1]

Etiology

It is postulated that the originating cell line for this disease is a mature (post-thymic) T-cell.^[1]

Signs and symptoms

Patients typically have systemic disease at presentation, including hepatosplenomegaly, generalized lymphadenopathy, and skin infiltrates.^[1]

Due to the systemic nature of this disease, leukemic cells can be found in peripheral blood, lymph nodes, bone marrow, spleen, liver, skin.^[1] A high lymphocyte count (> 100 x 10⁹/L)along with anemia and thrombocytopenia are common findings. HTLV-1 serologies are negative, and serum immunoglobins are within normal limits with no paraproteins present.^[1]

Morphology

In the peripheral blood, T-PLL consists of medium-sized lymphocytes with single nucleoli and basophilic cytoplasm with occasional blebs or projections. The nuclei are usually round to oval in shape, with occasional patients having cells with a more irregular nuclear outline that is similar to the cerebriform nuclear shape seen in Sézary syndrome.^[3] A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases.^[3]

Marrow involvement is typically diffuse with morphology similar to what is observed in peripheral blood.^[1] In the spleen, the leukemic cell infiltrate both the red pulp and white pulp, and lymph node involvement is typically diffuse through the paracortex.^[1] Skin infiltrates are seen in 20% of patients, and the infiltrates are usually dense and confined to the dermis and around the skin appendages.^[2]

Molecular findings

Immunophenotype

T-PLL has the immunophenotype of a mature (post-thymic) T-lymphocyte, and the neoplastic cells are typically positive for pan-T antigens CD2, CD3, and CD7 and negative for TdT and CD1a. The immunophenotype CD4+/CD8- is present in 60% of cases, the CD4+/CD8+ immunophenotype is present in 25%, and the CD4-/CD8+ immunophenotype is present in 15% of cases.^[2]

Genetic findings

Clonal TCR gene rearrangements for the γ and δ chains are typically found. The most frequent chromosomal abnormality is the inversion of chromosome 14, specifically inv 14(q11;q32). This is found in 80% of cases, while 10% of cases show a reciprocal translocation of chromosome 14 (t(14;14)(q11;q32)).^[4]

^[5] Also, abnormalities of chromosome 8 are seen approximately 75% of patients, including idic (8p11), t(8;8)(p11-12;q12), and trisomy 8. ^[6]

Treatment

Most patients with T-cell prolymphocytic leukemia require immediate treatment.^[7]

T-cell prolymphocytic leukemia is difficult to treat, and it does not respond to most available chemotherapeutic drugs.^[7] Many different treatments have been attempted, with limited success in certain patients: purine analogues (pentostatin, fludarabine, cladribine), chlorambucil, and various forms of combination chemotherapy regimens, including cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP), cyclophosphamide, vincristine, prednisone (COP), and vincristine, doxorubicin, prednisone, etoposide, cyclophosphamide, bleomycin (VAPEC-B).

Alemtuzumab (Campath), an anti-CD52 monoclonal antibody that attacks white blood cells, has been used in treatment with greater success than previous options.^[7] In one study of previously treated people with T-PLL, people who had a complete response to alemtuzumab survived a median of 16 months after treatment.^[7]

Some patients who successfully respond to treatment also undergo stem cell transplantation to consolidate the response.^[7]

Prognosis

T-PLL is an aggressive disease, and patients are not expected to live normal lifespans. Before the recent introduction of better treatments, such as alemtuzumab, the median survival time was 7.5 months after diagnosis.^[7] More recently, some patients have survived five years and more, although the median survival is still low.

Epidemiology

About four men are diagnosed with this disease for every three women.^[8] Despite its overall rarity, it is also the most common type of mature T cell leukemia.^[9]

References

^ ^a ^b ^c ^d ^e ^f ^g ^h Elaine Sarkin Jaffe, Nancy Lee Harris, World Health Organization, International Agency for Research on Cancer, Harald Stein, J.W. Vardiman (2001). Pathology and genetics of tumours of haematopoietic and lymphoid tissues. World Health Organization Classification of Tumors 3. Lyon: IARC Press. ISBN 92-832-2411-6.
^ ^a ^b ^c Matutes E, Brito-Babapulle V, Swansbury J, et al. (1991). "Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia". Blood 78 (12): 3269–74. PMID 1742486.
^ ^a ^b Matutes E, Garcia Talavera J, O'Brien M, Catovsky D (September 1986). "The morphological spectrum of T-prolymphocytic leukaemia". Br. J. Haematol. 64 (1): 111–24. doi:10.1111/j.1365-2141.1986.tb07579.x. PMID 3489482.
^ Brito-Babapulle V, Catovsky D (1991). "Inversions and tandem translocations involving chromosome 14q11 and 14q32 in T-prolymphocytic leukemia and T-cell leukemias in patients with ataxia telangiectasia". Cancer Genet. Cytogenet. 55 (1): 1–9. doi:10.1016/0165-4608(91)90228-M. PMID 1913594.
^ Maljaei SH, Brito-Babapulle V, Hiorns LR, Catovsky D (1998). "Abnormalities of chromosomes 8, 11, 14, and X in T-prolymphocytic leukemia studied by fluorescence in situ hybridization". Cancer Genet. Cytogenet. 103 (2): 110–6. doi:10.1016/S0165-4608(97)00410-X. PMID 9614908.
^ Sorour A, Brito-Babapulle V, Smedley D, Yuille M, Catovsky D (2000). "Unusual breakpoint distribution of 8p abnormalities in T-prolymphocytic leukemia: a study with YACS mapping to 8p11-p12". Cancer Genet. Cytogenet. 121 (2): 128–32. doi:10.1016/S0165-4608(00)00239-9. PMID 11063795.
^ ^a ^b ^c ^d ^e ^f Dearden CE, Matutes E, Cazin B, et al. (September 2001). "High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H". Blood 98 (6): 1721–6. doi:10.1182/blood.V98.6.1721. PMID 11535503.
^ Matutes Estella (1998). "T-cell prolymphocytic leukemia, a rare variant of mature post-thymic T-cell leukemias, has distinct clinical and laboratory characteristics and a poor prognosis". Cancer Control Journal 5 (1).
^ Valbuena JR, Herling M, Admirand JH, Padula A, Jones D, Medeiros LJ (March 2005). "T-cell prolymphocytic leukemia involving extramedullary sites". Am. J. Clin. Pathol. 123 (3): 456–64. doi:10.1309/93P4-2RNG-5XBG-3KBE. PMID 15716243.

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. T細胞性前リンパ性白血病の治療 treatment of t cell prolymphocytic leukemia
2. T細胞前リンパ球性白血病の臨床症状、病理学的特徴、および診断 clinical manifestations pathologic features and diagnosis of t cell prolymphocytic leukemia
3. B細胞前リンパ球性白血病 b cell prolymphocytic leukemia
4. Sézary症候群の臨床症状、病理学的特徴、および診断 clinical presentation pathologic features and diagnosis of sezary syndrome
5. 成人T細胞性白血病/リンパ腫の臨床症状、病理学的特徴、および診断 clinical manifestations pathologic features and diagnosis of adult t cell leukemia lymphoma

English Journal

Primary Refractory T-cell prolymphocytic leukaemia treated with Daily Administration of Alemtuzumab plus high dose methylprednisolone.

Renaudon-Smith E, Gribben JG, Agrawal SG.Author information Division of Haemato-Oncology, St Bartholomew's Hospital, Barts Health NHS Trust, London, UK.AbstractT cell prolymphocytic leukemia (T-PLL) is a rare lymphoproliferative disorder associated with an aggressive course and a poor response to conventional chemotherapy, with a median survival of 7.5.months1 . Non-chemotherapy treatment with the anti-CD52 monoclonal antibody, Alemtuzumab, is effective in the management of this condition, producing complete remissions in 81% of patients as first line treatment and 60% following relapse/refractoriness to other therapies2 . Currently there is no clear consensus in the literature about how to proceed in the setting of refractoriness to Alemtuzumab. One group has suggested that Pentostatin (a purine analogue) may be added, although this was in the setting of subcutaneous Alemtuzumab based on their prior experience with this as a single agent1 . This article is protected by copyright. All rights reserved.
European journal of haematology.Eur J Haematol.2013 Dec 14. doi: 10.1111/ejh.12248. [Epub ahead of print]
T cell prolymphocytic leukemia (T-PLL) is a rare lymphoproliferative disorder associated with an aggressive course and a poor response to conventional chemotherapy, with a median survival of 7.5.months1 . Non-chemotherapy treatment with the anti-CD52 monoclonal antibody, Alemtuzumab, is effective in
PMID 24329558

Minimal change disease in graft versus host disease: a podocyte response to the graft?

Huskey J, Rivard C, Myint H, Lucia S, Smith M, Shimada M, Ishimoto T, Araya C, Garin EH, Johnson RJ.AbstractNephrotic syndrome is a rare complication of hematopoietic cell transplantation. It has been suggested that nephrotic syndrome may represent a limited form of graft-versus-host disease although the pathological link between these two entities remains unclear. In this paper, we report a case of a 61-year-old female who underwent nonmyeloablative allogenic stem cell transplantation for T-cell prolymphocytic leukemia and subsequently developed biopsy proven minimal change disease shortly after cessation of her immunosuppression therapy. Urinary CD80 was markedly elevated during active disease and disappeared following corticosteroid-induced remission. We hypothesize that alloreactive donor T cells target the kidney and induce podocyte expression of CD80 that results in proteinuria from limited 'graft versus host' disease.
Clinical nephrology.Clin Nephrol.2013 Dec;80(6):469-73. doi: 10.5414/CN107420.
Nephrotic syndrome is a rare complication of hematopoietic cell transplantation. It has been suggested that nephrotic syndrome may represent a limited form of graft-versus-host disease although the pathological link between these two entities remains unclear. In this paper, we report a case of a 61-
PMID 23006339

Immunophenotypic characterization of T-cell prolymphocytic leukemia.

Chen X, Cherian S.Author information Dept of Laboratory Medicine, University of Washington, 1959 NE Pacific St, Seattle, WA 98195; xchen1@u.washington.edu.AbstractOBJECTIVES: To review clinical data, cytogenetic findings, and flow cytometric analysis in 20 patients with T-cell prolymphocytic leukemia (T-PLL), a rare, aggressive, mature T-cell leukemia with poor prognosis and short survival.
American journal of clinical pathology.Am J Clin Pathol.2013 Nov;140(5):727-35. doi: 10.1309/AJCPG71KYOXTKLQW.
OBJECTIVES: To review clinical data, cytogenetic findings, and flow cytometric analysis in 20 patients with T-cell prolymphocytic leukemia (T-PLL), a rare, aggressive, mature T-cell leukemia with poor prognosis and short survival.METHODS: Using multiparameter flow cytometry with a large combination
PMID 24124154

Japanese Journal

T-cell prolymphocytic leukemia in Japan : is it a variant?

KAMEOKA Junichi,TAKAHASHI Naoto,NOJI Hideyoshi,MURAI Kazunori,TAJIMA Katsushi,KAMEOKA Yoshihiro,SATO Shinji,SHICHISHIMA Tsutomu,ISHIDA Yoji,HARIGAE Hideo,SAWADA Kenichi
International journal of hematology 95(6), 660-667, 2012-06-01
NAID 10030793639

Epstein-Barr Virus-Negative Classical Hodgkin's Lymphoma in a Patient with T-Cell Prolymphocytic Leukemia Treated with Fludarabine

TSUJI Takahiro,YAMASAKI Hiroshi,ARIMA Nobuyuki,NIINO Daisuke,OHSHIMA Koichi,TSUDA Hiroyuki
Journal of clinical and experimental hematopathology 52(1), 71-75, 2012-05-01
NAID 10030615686

TCL1A gene involvement in T-cell prolymphocytic leukemia in Japanese patients

YOKOHAMA Akihiko,SAITOH Akio,NAKAHASHI Hirotaka,MITSUI Takeki,KOISO Hiromi,KIM Yoshitora,UCHIUMI Hideki,SAITOH Takayuki,HANDA Hiroshi,JIMBO Takahiro,MURAYAMA Kayoko,SAKURA Tohru,MURAKAMI Hirokazu,KARASAWA Masamitsu,NOJIMA Yoshihisa,TSUKAMOTO Norifumi
International journal of hematology 95(1), 77-85, 2012-01-01
NAID 10030266898

Related Pictures

★リンクテーブル★

リンク元	「T細胞性前リンパ性白血病」「T細胞性前リンパ球性白血病」「T-cell prolymphocytic leukaemia」「T-PLL」
関連記事	「T」「prolymphocytic」「cell」