Tumors of the hematopoietic and lymphoid tissues










Tumors of the hematopoietic and lymphoid tissues
Plasmacytoma ultramini1.jpg

Micrograph of a plasmacytoma, a hematological malignancy

Tumors of the hematopoietic and lymphoid tissues (American English) or tumours of the haematopoietic and lymphoid malignancies (British English) are tumors that affect the blood, bone marrow, lymph, and lymphatic system.[1][2] As those elements are all intimately connected through both the circulatory system and the immune system, a disease affecting one will often affect the others as well, making myeloproliferation and lymphoproliferation (and thus the leukemias and the lymphomas) closely related and often overlapping problems.


While uncommon in solid tumors, chromosomal translocations are a common cause of these diseases. This commonly leads to a different approach in diagnosis and treatment of haematological malignancies.


Haematological malignancies are malignant neoplasms ("cancer"), and they are generally treated by specialists in hematology and/or oncology. In some centers "haematology/oncology" is a single subspecialty of internal medicine while in others they are considered separate divisions (there are also surgical and radiation oncologists). Not all haematological disorders are malignant ("cancerous"); these other blood conditions may also be managed by a hematologist.


Hematological malignancies may derive from either of the two major blood cell lineages: myeloid and lymphoid cell lines. The myeloid cell line normally produces granulocytes, erythrocytes, thrombocytes, macrophages and mast cells; the lymphoid cell line produces B, T, NK and plasma cells. Lymphomas, lymphocytic leukemias, and myeloma are from the lymphoid line, while acute and chronic myelogenous leukemia, myelodysplastic syndromes and myeloproliferative diseases are myeloid in origin.


A subgroup of them are more severe and are known as haematological malignancies (British English)/hematological malignancies (American English) or blood cancer. They may also be referred to as liquid tumors.[3][4]




Contents






  • 1 Diagnosis


    • 1.1 Classification


      • 1.1.1 Classification according to WHO






  • 2 Treatment


  • 3 Follow-up


  • 4 Epidemiology


  • 5 See also


  • 6 References


  • 7 External links





Diagnosis


For the analysis of a suspected hematological malignancy, a complete blood count and blood film are essential, as malignant cells can show in characteristic ways on light microscopy. When there is lymphadenopathy, a biopsy from a lymph node is generally undertaken surgically. In general, a bone marrow biopsy is part of the "work up" for the analysis of these diseases. All specimens are examined microscopically to determine the nature of the malignancy. A number of these diseases can now be classified by cytogenetics (AML, CML) or immunophenotyping (lymphoma, myeloma, CLL) of the malignant cells.



Classification


Historically, hematological malignancies have been most commonly divided by whether the malignancy is mainly located in the blood (leukemia) or in lymph nodes (lymphomas).


However, the influential WHO Classification (published in 2001) placed a greater emphasis on cell lineage.


Relative proportions of hematological malignancies in the United States[5]




































































Type of hematological malignancy
 Percentage
 Total

Leukemias
 —
 30.4%

Acute lymphoblastic leukemia (ALL)
 4.0%

Acute myelogenous leukemia (AML)
 8.7%

Chronic lymphocytic leukemia (CLL)
sorted under lymphomas according to current WHO classification; called small lymphocytic lymphoma (SLL) when leukemic cells are absent.
 10.2%

Chronic myelogenous leukemia (CML)


Acute monocytic leukemia (AMoL)
 0.7%
Other leukemias
 3.1%

Lymphomas
 —
 55.6%

Hodgkin's lymphomas (all four subtypes)
 7.0%

Non-Hodgkin's lymphomas (all subtypes)
 48.6%

Myelomas
14.0%
Total
100%


Classification according to WHO


4th Edition[6]


NOS = "Not otherwise specified"




  • myeloid neoplasms
















    • Blastic plasmacytoid dendritic cell neoplasm






  • lymphoid neoplasms

















Treatment


Treatment can occasionally consist of "watchful waiting" (e.g. in CLL) or symptomatic treatment (e.g. blood transfusions in MDS). The more aggressive forms of disease require treatment with chemotherapy, radiotherapy, immunotherapy and—in some cases—a bone marrow transplant. The use of rituximab has been established for the treatment of B-cell–derived hematologic malignancies, including follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL).[7]



Follow-up


If treatment has been successful ("complete" or "partial remission"), a person is generally followed up at regular intervals to detect recurrence and monitor for "secondary malignancy" (an uncommon side-effect of some chemotherapy and radiotherapy regimens—the appearance of another form of cancer). In the follow-up, which should be done at pre-determined regular intervals, general anamnesis is combined with complete blood count and determination of lactate dehydrogenase or thymidine kinase in serum.



Epidemiology


Taken together, haematological malignancies account for 9.5% of new cancer diagnoses in the United States[8] and 30,000 patients in the UK are diagnosed each year.[9] Within this category, lymphomas are more common than leukemias.



See also


  • Myelodysplastic–myeloproliferative diseases


References





  1. ^ Vardiman, JW; Thiele, J; Arber, DA; Brunning, RD; Borowitz, MJ; Porwit, A; Harris, NL; Le Beau, MM; Hellström-Lindberg, E; Tefferi, A; Bloomfield, CD (30 July 2009). "The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes". Blood. 114 (5): 937–51. doi:10.1182/blood-2009-03-209262. PMID 19357394..mw-parser-output cite.citation{font-style:inherit}.mw-parser-output .citation q{quotes:"""""""'""'"}.mw-parser-output .citation .cs1-lock-free a{background:url("//upload.wikimedia.org/wikipedia/commons/thumb/6/65/Lock-green.svg/9px-Lock-green.svg.png")no-repeat;background-position:right .1em center}.mw-parser-output .citation .cs1-lock-limited a,.mw-parser-output .citation .cs1-lock-registration a{background:url("//upload.wikimedia.org/wikipedia/commons/thumb/d/d6/Lock-gray-alt-2.svg/9px-Lock-gray-alt-2.svg.png")no-repeat;background-position:right .1em center}.mw-parser-output .citation .cs1-lock-subscription a{background:url("//upload.wikimedia.org/wikipedia/commons/thumb/a/aa/Lock-red-alt-2.svg/9px-Lock-red-alt-2.svg.png")no-repeat;background-position:right .1em center}.mw-parser-output .cs1-subscription,.mw-parser-output .cs1-registration{color:#555}.mw-parser-output .cs1-subscription span,.mw-parser-output .cs1-registration span{border-bottom:1px dotted;cursor:help}.mw-parser-output .cs1-ws-icon a{background:url("//upload.wikimedia.org/wikipedia/commons/thumb/4/4c/Wikisource-logo.svg/12px-Wikisource-logo.svg.png")no-repeat;background-position:right .1em center}.mw-parser-output code.cs1-code{color:inherit;background:inherit;border:inherit;padding:inherit}.mw-parser-output .cs1-hidden-error{display:none;font-size:100%}.mw-parser-output .cs1-visible-error{font-size:100%}.mw-parser-output .cs1-maint{display:none;color:#33aa33;margin-left:0.3em}.mw-parser-output .cs1-subscription,.mw-parser-output .cs1-registration,.mw-parser-output .cs1-format{font-size:95%}.mw-parser-output .cs1-kern-left,.mw-parser-output .cs1-kern-wl-left{padding-left:0.2em}.mw-parser-output .cs1-kern-right,.mw-parser-output .cs1-kern-wl-right{padding-right:0.2em}


  2. ^ World Cancer Report 2014. World Health Organization. 2014. pp. Chapter 5.13. ISBN 9283204298.


  3. ^ Juo, Pei-Show (2001). Concise Dictionary of Biomedicine and Molecular Biology (2nd ed.). Hoboken: CRC Press. p. 653. ISBN 9781420041309.


  4. ^ Cancer Rehabilitation Medicine Quick Reference (RMQR). New York: Demos Medical Publishing. 2013. p. 26. ISBN 9781617050008.


  5. ^ Horner MJ, Ries LAG, Krapcho M, Neyman N, et al. (eds). "SEER Cancer Statistics Review, 1975–2006". Surveillance Epidemiology and End Results (SEER). Bethesda, MD: National Cancer Institute. Retrieved 3 November 2009. Table 1.4: Age-Adjusted SEER Incidence and U.S. Death Rates and 5-Year Relative Survival Rates By Primary Cancer Site, Sex and Time PeriodCS1 maint: Multiple names: authors list (link)


  6. ^ al.], edited by Steven H. Swerdlow ... [et (2008). WHO classification of tumours of haematopoietic and lymphoid tissues (4th. ed.). Lyon, France: International Agency for Research on Cancer. p. 10. ISBN 9283224310.CS1 maint: Extra text: authors list (link)


  7. ^ "The Clinical and Economic Value of Rituximab for the Treatment of Hematologic Malignancies". Contemporary Oncology. Retrieved 14 September 2011.


  8. ^ "Facts & Statistics". The Leukemia and Lymphoma Society. Archived from the original on 27 May 2010. Retrieved 3 November 2009.


  9. ^ "Facts about blood cancers". Leukaemia & Lymphoma Research. Retrieved 24 September 2013.




External links





Classification
D



  • ICD-10: C81-C96


  • ICD-9-CM: 200-209


  • ICD-O: 9590-9999


  • MeSH: D019337









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