Tumors of the hematopoietic and lymphoid tissues (American English) or tumours of the haematopoietic and lymphoid tissues (British English) are tumors that affect the blood, bone marrow, lymph, and lymphatic system.[1][2] Because these tissues 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 aplasia, 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 hematological malignancies.
Hematological malignancies are malignant neoplasms ("cancer"), and they are generally treated by specialists in hematology and/or oncology. In some centers "hematology/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 hematological disorders are malignant ("cancerous"); these other blood conditions may also be managed by a hematologist.
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]
Diagnosisedit
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.[citation needed]
Classificationedit
Historically, hematological malignancies have been most commonly divided by whether the malignancy is mainly located in the blood (leukemia) or in lymph nodes (lymphomas).
Relative proportions of hematological malignancies in the United States[5]
Chronic lymphocytic leukemia (CLL) sorted under lymphomas according to current WHO classification; called small lymphocytic lymphoma (SLL) when leukemic cells are absent.
Other iatrogenic immunodeficiency- associated lymphoproliferative disorders
Histiocytic and dendritic cell neoplasms
Histiocytic sarcoma
Langerhans cell histiocytosis, NOS
Langerhans cell histiocytosis, monostotic
Langerhans cell histiocytosis, polystotic
Langerhans cell histiocytosis, disseminated
Langerhans cell sarcoma
Indeterminate dendritic cell tumour
Interdigitating dendritic cell sarcoma
Follicular dendritic cell sarcoma
Fibroblastic reticular cell tumour
Disseminated juvenile xanthogranuloma
Erdheim–Chester disease
Treatmentedit
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]
In addition to cure-directed treatment, people can benefit from self-care to manage symptoms. For example, aerobic exercise, such as walking, can reduce fatigue and feelings of depression in people with hematological malignancies.[8]
Follow-upedit
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. Hematological malignancies as well as their treatments are associated with complications affecting many organs, with the lungs being frequently affected[9][10]
Taken together, haematological malignancies account for 9.5% of new cancer diagnoses in the United States[13] and 30,000 patients in the UK are diagnosed each year.[14] Within this category, lymphomas are more common than leukemias.[citation needed]
^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. S2CID 3101472.
^World Cancer Report 2014. World Health Organization. 2014. pp. Chapter 5.13. ISBN 978-9283204299.
^Juo, Pei-Show (2001). Concise Dictionary of Biomedicine and Molecular Biology (2nd ed.). Hoboken: CRC Press. p. 653. ISBN 9781420041309.
^Cancer Rehabilitation Medicine Quick Reference (RMQR). New York: Demos Medical Publishing. 2013. p. 26. ISBN 9781617050008.
^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 Period{{cite web}}: CS1 maint: multiple names: authors list (link)
^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 978-9283224310. {{cite book}}: |first1= has generic name (help)
^"The Clinical and Economic Value of Rituximab for the Treatment of Hematologic Malignancies". Spring 2011. Contemporary Oncology. 15 March 2011. Retrieved 14 September 2011.
^Knips, Linus; Bergenthal, Nils; Streckmann, Fiona; Monsef, Ina; Elter, Thomas; Skoetz, Nicole (31 January 2019). "Aerobic physical exercise for adult patients with haematological malignancies". The Cochrane Database of Systematic Reviews. 1 (1): CD009075. doi:10.1002/14651858.CD009075.pub3. ISSN 1469-493X. PMC6354325. PMID 30702150.
^Br J Hosp Med (Lond). 2014 Dec;75(12):691–7.
Non-infectious respiratory disease in non-HIV immunocompromised patients.
Jose RJ1, Faiz SA, Dickey BF, Brown JS. doi:10.12968/hmed.2014.75.12.691.
^Br J Hosp Med (Lond). 2014 Dec;75(12):685–90.
Infectious respiratory disease in non-HIV immunocompromised patients.
Jose RJ1, Dickey BF, Brown JS. PMID 25488531 doi:10.12968/hmed.2014.75.12.685
^ abValikhani M, Rahimian E, Ahmadi SE, Chegeni R, Safa M. Involvement of classic and alternative non-homologous end joining pathways in hematologic malignancies: targeting strategies for treatment. Exp Hematol Oncol. 2021 Nov 3;10(1):51. doi: 10.1186/s40164-021-00242-1. PMID 34732266; PMCID: PMC8564991
^Senapati J, Sasaki K. Chromosomal Instability in Chronic Myeloid Leukemia: Mechanistic Insights and Effects. Cancers (Basel). 2022 May 21;14(10):2533. doi: 10.3390/cancers14102533. PMID 35626137; PMCID: PMC9140097