| Hematological malignancy |
| Classification and external resources |
Micrograph of a plasmacytoma, a hematological malignancy. |
| ICD-10 |
C81-C96 |
| ICD-9 |
200-208 |
| ICD-O: |
9590-9999 |
| MeSH |
D019337 |
Hematological malignancies are the types of cancer that affect blood, bone marrow, and lymph nodes. As the three are intimately connected through the immune system, a disease affecting one of the three will often affect the others as well: although lymphoma is technically a disease of the lymph nodes, it often spreads to the bone marrow, affecting the blood and occasionally producing a paraprotein.
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.
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.
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Contents
- 1 Classification and incidence
- 1.1 Classic classification
- 1.1.1 Relative proportions of hematological malignancies in the United States:[2]
- 1.2 By cell lineage
- 2 Diagnosis
- 3 Treatment
- 4 Follow-up
- 5 References
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Classification and incidence
Taken together, hematological malignancies account for 9.5% of new cancer diagnoses in the United States.[1] Within this category, lymphomas are more common than leukemias.
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) emphasized a greater emphasis on cell lineage.
Classic classification
Relative proportions of hematological malignancies in the United States:[2]
| 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) |
3.7% |
|
| 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% |
By cell lineage
For extensive list, see navigation bars at article bottom
By cell lineage, hematological malignancies are classified according to their lineage in hematopoiesis:
-
- Lymphoid leukemia
- Lymphoma
- Myeloid leukemia
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.
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) [3]
Follow-up
If treatment has been successful ("complete" or "partial remission"), a patient 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.
References
- ^ "Facts & Statistics". The Leukemia and Lymphoma Society. http://www.leukemia-lymphoma.org/all_page?item_id=12486. Retrieved 03 November 2009.
- ^ 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. http://seer.cancer.gov/csr/1975_2006/. Retrieved 03 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"
- ^ "The Clinical and Economic Value of Rituximab for the Treatment of Hematologic Malignancies". Contemporary Oncology. http://www.onclive.com/publications/contemporary-oncology/2011/spring-2011/the-clinical-and-economic-value-of-rituximab-for-the-treatment-of-hematologic-malignancies. Retrieved 14 September 2011.
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Hematological malignancy/leukemia histology (ICD-O 9590–9989, C81–C96, 200–208)
Lymphoid/Lymphoproliferative, Lymphomas/Lymphoid leukemias (9590–9739, 9800–9839)
|
|
B cell
(lymphoma,
leukemia)
(most CD19, CD20) |
|
By development/
marker
|
|
TdT+
|
ALL (Precursor B acute lymphoblastic leukemia/lymphoma)
|
|
|
CD5+
|
naive B cell (CLL/SLL)
mantle zone (Mantle cell)
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CD22+
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Prolymphocytic · CD11c (Hairy cell leukemia)
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CD79a+
|
germinal center/follicular B cell (Follicular, Burkitt's, GCB DLBCL, Primary cutaneous follicular lymphoma)
marginal zone/marginal-zone B cell (Splenic marginal zone, MALT, Nodal marginal zone, Primary cutaneous marginal zone lymphoma)
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RS (CD15+, CD30+)
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Classic Hodgkin's lymphoma (Nodular sclerosis) · CD20 (Nodular lymphocyte predominant Hodgkin's lymphoma)
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PCDs/PP
(CD38+/CD138+)
|
see immunoproliferative immunoglobulin disorders
|
|
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By infection
|
KSHV (Primary effusion) · EBV (Lymphomatoid granulomatosis, Post-transplant lymphoproliferative disorder) · HIV (AIDS-related lymphoma) · Helicobacter pylori (MALT lymphoma)
|
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Cutaneous
|
Diffuse large B-cell lymphoma · Intravascular large B-cell lymphoma · Primary cutaneous marginal zone lymphoma · Primary cutaneous immunocytoma · Plasmacytoma · Plasmacytosis · Primary cutaneous follicular lymphoma
|
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|
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T/NK
|
T cell
(lymphoma,
leukemia)
(most CD3, CD4, CD8) |
|
By development/
marker
|
TdT+: ALL (Precursor T acute lymphoblastic leukemia/lymphoma)
prolymphocyte (Prolymphocytic)
CD30+ (Anaplastic large-cell lymphoma, Lymphomatoid papulosis type A)
|
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Cutaneous
|
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MF+variants
|
indolent: Mycosis fungoides · Pagetoid reticulosis · Granulomatous slack skin
aggressive: Sézary's disease · Adult T-cell leukemia/lymphoma
|
|
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Non-MF
|
CD30-: Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma · Pleomorphic T-cell lymphoma · Lymphomatoid papulosis type B
CD30+: CD30+ cutaneous T-cell lymphoma · Secondary cutaneous CD30+ large cell lymphoma · Lymphomatoid papulosis type A
|
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Other peripheral
|
Hepatosplenic · Angioimmunoblastic · Enteropathy-associated T-cell lymphoma · Peripheral T-cell lymphoma-Not-Otherwise-Specified (Lennert lymphoma) · Subcutaneous T-cell lymphoma
|
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By infection
|
HTLV-1 (Adult T-cell leukemia/lymphoma)
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NK cell/
(most CD56)
|
Aggressive NK-cell leukemia · Blastic NK cell lymphoma
|
|
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T or NK
|
EBV (Extranodal NK-T-cell lymphoma/Angiocentric lymphoma) · Large granular lymphocytic leukemia
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Lymphoid+myeloid
|
Acute biphenotypic leukaemia
|
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Lymphocytosis
|
Lymphoproliferative disorders (X-linked lymphoproliferative disease, Autoimmune lymphoproliferative syndrome) · Leukemoid reaction · Diffuse infiltrative lymphocytosis syndrome
|
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| Cutaneous lymphoid hyperplasia |
Cutaneous lymphoid hyperplasia with bandlike and perivascular patterns · Cutaneous lymphoid hyperplasia with nodular pattern · Jessner lymphocytic infiltrate of the skin
|
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cell/phys/auag/auab/comp, igrc
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Myeloid hematological malignancy/leukemia histology (ICD-O 9590–9989, C81–C96, 200–208)
|
|
CFU-GM/
and other granulocytes |
|
CFU-GM
|
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Myelocyte
|
AML: Acute myeloblastic leukemia (M0, M1, M2), APL/M3
MP (Chronic neutrophilic leukemia)
|
|
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Monocyte
|
AML (AMoL/M5, Myeloid dendritic cell leukemia)
CML (Philadelphia chromosome, Accelerated phase chronic myelogenous leukemia)
|
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Myelomonocyte
|
AML (M4)
MD-MP (Juvenile myelomonocytic leukemia, Chronic myelomonocytic leukemia)
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Other
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Histiocytosis
|
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CFU-Baso
|
AML (Acute basophilic)
|
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CFU-Eos
|
AML (Acute eosinophilic)
MP (Chronic eosinophilic leukemia/Hypereosinophilic syndrome)
|
|
|
| MEP |
|
CFU-Meg
|
AML (AMKL/M7)
MP (Essential thrombocytosis)
|
|
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CFU-E
|
AML (Erythroleukemia/M6)
MP (Polycythemia vera)
MD (Refractory anemia, Refractory anemia with excess of blasts, Chromosome 5q deletion syndrome, Sideroblastic anemia, Paroxysmal nocturnal hemoglobinuria, Refractory cytopenia with multilineage dysplasia)
|
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| CFU-Mast |
Mastocytoma (Mast cell leukemia, Mast cell sarcoma, Systemic mastocytosis)
Mastocytosis: Diffuse cutaneous mastocytosis · Erythrodermic mastocytosis · Generalized eruption of cutaneous mastocytosis (adult type) · Generalized eruption of cutaneous mastocytosis (childhood type) · Mast cell sarcoma · Solitary mastocytoma · Systemic mastocytosis · Xanthelasmoidal mastocytosis
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| Multiple/unknown |
AML (Acute panmyelosis with myelofibrosis, Myeloid sarcoma) · MP (Myelofibrosis) · Acute biphenotypic leukaemia
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cell/phys (coag, heme, immu, gran), csfs
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rbmg/mogr/tumr/hist, sysi/epon, btst
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drug (B1/2/3+5+6), btst, trns
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