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.
Chromosomal translocations are a common cause of these diseases, while this is uncommon in solid tumors. This leads to a different approach in diagnosis and treatment of hematological malignancies.
Although hematological malignancies are a form of cancer, they are generally treated by specialists in hematology, although in many hospitals oncology specialists also manage these diseases. ("Hematology/Oncology" is a single subspecialty of Internal Medicine; there are also surgical and radiation oncologists.)
There are two normal cell lineages from which hematological malignancies derive, myeloid and lymphoid. The former normally produces granulocytes, erythrocytes, thrombocytes, macrophages and mast cells, the latter B, T, NK and plasma cells. Lymphomas, acute lymphoblastic leukemia and myeloma are from the lymphoid line, while acute and chronic myelogenous leukemia, myelodysplastic syndromes and myeloproliferative diseases are myeloid in origin.
Chromosomal translocations are a common cause of these diseases, while this is uncommon in solid tumors. This leads to a different approach in diagnosis and treatment of hematological malignancies.
Although hematological malignancies are a form of cancer, they are generally treated by specialists in hematology, although in many hospitals oncology specialists also manage these diseases. ("Hematology/Oncology" is a single subspecialty of Internal Medicine; there are also surgical and radiation oncologists.)
There are two normal cell lineages from which hematological malignancies derive, myeloid and lymphoid. The former normally produces granulocytes, erythrocytes, thrombocytes, macrophages and mast cells, the latter B, T, NK and plasma cells. Lymphomas, acute lymphoblastic leukemia and myeloma are from the lymphoid line, while acute and chronic myelogenous leukemia, myelodysplastic syndromes and myeloproliferative diseases are myeloid in origin.
List of diseases
The hematological malignancies include:
Leukemia:
Acute lymphoblastic leukemia (ALL)
Acute myelogenous leukemia (AML)
Chronic myelogenous leukemia (CML)
Lymphoma:
Hodgkin's disease (four subtypes)
Non-Hodgkin lymphoma - many subtypes, including
Chronic lymphocytic leukemia (CLL) / Small lymphocytic lymphoma (SLL)
Diffuse large B-cell lymphoma (DLBCL)
Follicular lymphoma (FL)
Mantle cell lymphoma (MCL)
Hairy cell leukemia (HCL)
Marginal zone lymphoma (MZL)
Burkitt's lymphoma (BL)
Post-transplant lymphoproliferative disorder (PTLD)
T-cell prolymphocytic leukemia (T-PLL)
B-cell prolymphocytic leukemia (B-PLL)
Waldenström's macroglobulinemia / Lymphoplasmacytic lymphoma
Other NK- or T-cell lymphomas (several types)
Multiple myeloma
Related disorders, which are generally not called "cancer":
Myelodysplastic syndrome (MDS) - can culminate in AML
Myeloproliferative disease:
Polycythemia vera (PV, PCV or occasionally polycythemia rubra vera - PRV)
Essential thrombocytosis (ET)
Myelofibrosis
MDS/MPD (diseases with features of both disorders):
Chronic myelomonocytic leukemia (CMML)
Juvenile myelomonocytic leukemia (JMML)
Atypical chronic myeloid leukemia (aCML)
Myelodysplastic/myeloproliferative disease, unclassifiable (MDS/MPD, U)
Provisional entity: Refractory anemia with ringed sideroblasts (RARS) associated with marked thrombocytosis
Amyloid due to light-chain disease
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.
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.
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