plasma cell leukemia

WordNet

1. small room in which a monk or nun lives (同)cubicle
2. a device that delivers an electric current as the result of a chemical reaction (同)electric cell
3. a room where a prisoner is kept (同)jail cell, prison cell
4. (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
5. any small compartment; "the cells of a honeycomb"
6. a small unit serving as part of or as the nucleus of a larger political movement (同)cadre
7. (physical chemistry) a fourth state of matter distinct from solid or liquid or gas and present in stars and fusion reactors; a gas becomes a plasma when it is heated until the atoms lose all their electrons, leaving a highly electrified collection of nuclei and free electrons; "particles in space exist in the form of a plasma"
8. a green slightly translucent variety of chalcedony used as a gemstone
9. the colorless watery fluid of the blood and lymph that contains no cells, but in which the blood cells (erythrocytes, leukocytes, and thrombocytes) are suspended (同)plasm, blood_plasma
10. 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

PrepTutorEJDIC

1. (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
2. (また『blood plasma』)血漿,リンパ漿 / 原形質
3. 白血病

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/11/21 15:13:41」(JST)

wiki en

[Wiki en表示]

Plasma cell leukemia
A schematic showing peripheral blood with plasma cell leukemia. Many plasma cells are seen mixed with red cells.
Classification and external resources
Specialty	Hematology and oncology
ICD-10	C90.1
ICD-9-CM	203.1
ICD-O	M9733/3
MeSH	D007952

Plasma cell leukemia (PCL), a lymphoproliferative disorder, is a rare cancer involving a subtype of white blood cells called plasma cells. Plasma cell leukemia is one of the most aggressive human neoplasms and constitutes 2% to 4% of all cases of plasma cell disorders. The WHO criterion for diagnosis of PCL is that plasma cells constitute more than 20% of cells in the peripheral blood with an absolute plasma cell count of more than 2 × 10⁹/L. The presentation may be primary, de novo, or secondary, evolving from an existing case of multiple myeloma as part of the terminal phase of the disease. Approximately 60 to 70% of cases are primary.

Laboratory findings

Although there is overlap, the markers of plasma cells from patients with primary PCL is different from those of myeloma patients. PCL plasma cells more frequently express the CD20 antigen than those of multiple myeloma (50% vs. 17%), and they often lack CD56 antigen which is present on the majority of myeloma cells. CD56 is considered important in anchoring plasma cells to bone marrow stroma, and its expression is associated with a poor prognosis. CD28 is more frequently expressed on malignant plasma cells in secondary than in primary PCL, which is consistent with an observation made in myeloma, that is, that acquisition of the CD28 antigen on plasma cells appears to correlate with an increased proliferative rate and disease progression.

The chromosome characteristics or karyotypes of PCL plasma cells is complex. Using fluorescence in situ hybridization techniques, losses in the long arm of chromosome 13 (13q) and deletion of one the homologous chromosomes 13 (monosmy 13) exist in more than 80% of PCL patients. Losses on chromosome 16 also occur in approximately 80% of cases. Gains in long arm of chromosome 1 (1q) are present in approximately one-half of the patients determined by FISH, but in all patients by comparative genomic hybridization. Overexpression of the protein PRAD1/cyclin D1, which plays an important role in control of the cell cycle, has also been observed in PCL. Plasma cell leukemia is more frequent in light-chain (Bence Jones protein) or IgD myeloma. It is seen less frequently in IgA or IgG myeloma (see: multiple myeloma).

Clinical course

The clinical presentation of primary PCL is more aggressive than that of multiple myeloma, and the clinical features found in PCL involves combination of those found in acute leukemia and those found in myeloma. PCL patients have a higher presenting tumor burden; higher frequencies of (involvement of organs other than the bone marrow by disease), thrombocytopenia (decreased platelet count), anemia, hypercalcemia (elevated blood calcium), and renal impairment; increased levels of serum lactate dehydrogenase (LDH) and β-2 macroglobulin and plasma cell proliferative activity. The incidence of lytic bone lesions is slightly lower than that usually observed in multiple myeloma. PCL is extremely aggressive associated with short survival (median survival of 6–8 months).

Therapy and prognosis

Treatment of plasma cell leukemia is by supportive care and systemic chemotherapy. Combination of cyclophosphamide, doxorubicin, vincristine, and prednisone (or dexamethasone) can be used. A second combination is the use of cyclophosphamide, dexamethasone, and thalidomide as for myeloma. Another regimen termed VMCP/VBAP uses alternating vincristine, melphalan (M), cyclophosphamide, prednisone/vincristine, carmustine, doxorubicin, and prednisone polychemotherapy. In general, combination chemotherapy has resulted in median survivals of 18 to 20 months compared to 2 to 6 months when single agent therapy is used. There are anecdotal reports of excellent responses and 2- to 3-year disease-free survivals after autologous stem cell transplantation. Autologous transplantation may increase benefit from conventional therapy and high dose melphalan may prolong progression free and overall survival.

Although the clinical and laboratory features of primary and secondary PCL are similar, the response to therapy and overall survival in primary and secondary PCL go from poor to worse. Patients with secondary PCL are usually refractory to chemotherapy and have a poor survival compared with duration (median <2 months).

Novel agents like thalidomide, lenalidomide and bortezomib have been recently used, alone or in combination with conventional chemotherapeutics like melphalan or cyclophosphamide. Two cases had encouraging results with bortezomib. Recent studies demonstrate that velcade (bortezomib)- based regimens offer a significant profit in patients suffering from PCL. More aggressive therapeutic regimens like DT-PACE or protocols used in ALL offer a significant response rate, but with short remission interval.

References

Bibliography

• Greer JP, Foerster J, and Lukens JN, "Wintrobe's Clinical Hematology", Lippincott Williams & Wilkins 11th ed., 2003.
• Hoffman R, Benz E, Shattil S, Furie B, Cohen H, "Hematology: Basic Principles and Practice", Churchill Livingstone, 4th ed, 2004.
• Hoffbrand AV, Catovsky D, and Tuddenham E, "Postgraduate Haematology", Blackwell, 5th ed., 2005.
• Hoffbrand AV, Moss PAH, and Pettit JE, "Essential Haematology", Blackwell, 5th ed., 2006.

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. 形質細胞白血病 plasma cell leukemia
• 2. 多発性骨髄腫の臨床的特徴、検査所見、および診断 clinical features laboratory manifestations and diagnosis of multiple myeloma
• 3. 多発性骨髄腫における病期分類および予後研究 staging and prognostic studies in multiple myeloma
• 4. 多発性骨髄腫の病理生物学 pathobiology of multiple myeloma
• 5. 多発性骨髄腫患者のマネージメントの概要 overview of the management of multiple myeloma

English Journal

• Alcohol consumption and risk of lymphoid and myeloid neoplasms: Results of the Netherlands cohort study.

• Heinen MM, Verhage BA, Schouten LJ, Goldbohm RA, Schouten HC, van den Brandt PA.SourceSchool for Oncology and Developmental Biology (GROW), Department of Epidemiology, Maastricht University Medical Center, Maastricht, The Netherlands.
• International journal of cancer. Journal international du cancer.Int J Cancer.2013 Oct 1;133(7):1701-12. doi: 10.1002/ijc.28175. Epub 2013 Apr 24.
• Results from epidemiological studies suggest that alcohol drinkers have a decreased risk of lymphoid neoplasms, whereas results for myeloid neoplasms are inconsistent. However, most of these studies have used retrospective data. We examined prospectively whether alcohol consumption decreases the ris
• PMID 23553592

• Anti-proliferative actions of N'-desmethylsorafenib in human breast cancer cells.

• Cui PH, Rawling T, Gillani TB, Bourget K, Wang XS, Zhou F, Murray M.SourcePharmacogenomics and Drug Development Group, Discipline of Pharmacology, University of Sydney, NSW 2006, Australia; Faculty of Pharmacy, University of Sydney, NSW 2006, Australia.
• Biochemical pharmacology.Biochem Pharmacol.2013 Aug 1;86(3):419-27. doi: 10.1016/j.bcp.2013.05.014. Epub 2013 May 31.
• The multi-kinase inhibitor sorafenib is used for the treatment of renal and hepatic carcinomas and is undergoing evaluation for treatment of breast cancer in combination with other agents. Cytochrome P450 (CYP) 3A4 converts sorafenib to multiple metabolites that have been detected in patient plasma.
• PMID 23732299

• Extracellular vesicles in chronic lymphocytic leukemia.

• Haderk F, Hanna B, Richter K, Schnölzer M, Zenz T, Stilgenbauer S, Lichter P, Seiffert M.SourceDepartment of Molecular Genetics, German Cancer Research Center (DKFZ) , Heidelberg , Germany.
• Leukemia & lymphoma.Leuk Lymphoma.2013 Aug;54(8):1826-30. doi: 10.3109/10428194.2013.796052.
• Abstract Extracellular vesicles (EVs) are membrane-enclosed nanoparticles 30 to 1000 nm in size and represent a novel mechanism of cell communication. By transferring RNA and protein from their cell of origin, they can reprogram target cells and thus are involved in changes within the cellular micro
• PMID 23848063

Japanese Journal

• 形質細胞性白血病の診断と治療 (造血器腫瘍学 : 基礎と臨床の最新研究動向) -- (リンパ系腫瘍の臨床)

• 三輪 哲義
• 日本臨床 70 (1018 増刊2), 628-641, 2012-04
• NAID 40019282999

• 症例報告 ボルテゾミブが著効した重度の高ビリルビン血症を合併した形質細胞性白血病

• 美濃 達治,坂井 晃,木下 未来 [他]
• 臨床血液 53(1), 92-96, 2012-01
• NAID 40019188435

Related Links

• Plasma cell leukemia - Wikipedia, the free encyclopedia

Plasma cell leukemia (PCL), a lymphoproliferative disorder, is a rare cancer involving a subtype of white blood cells called plasma cells. Plasma cell leukemia is one of the most aggressive human neoplasms and constitutes 2% to 4% of all ...

• Plasma cell leukemia - Haematologica

1 May 2010 ... In this issue of Haematologica, Drake et al. report on a very large number of patients with plasma cell leukemia abstracted from the European Group for Blood and Marrow Transplantation.1 Having analyzed data from 272 ...

Related Pictures

---

★リンクテーブル★

リンク元	「plasmacytic leukemia」
関連記事	「plasma cell」「cell」

「plasmacytic leukemia」

　　[★]

形質細胞性白血病

関

plasma cell leukemia

「plasma cell」

　　[★] 形質細胞

同

plasma cells, in peripheral blood smeare

「cell」

　　[★] 細胞