Hematological malignancy

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.

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.

Hematological malignancies are malignant neoplasms ("cancer"), and they are generally treated by specialists in hematology and/or oncology. "Hematology/oncology" is a single subspecialty of internal medicine (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.

List of diseases

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.

Relative proportions of hematoloical malignancies in the United States^[2]

There are many subtypes of Non-Hodgkin lymphoma, including:

• Diffuse large B-cell lymphoma (DLBCL)
• Follicular lymphoma (FL)
• Mantle cell lymphoma (MCL)
• Marginal zone lymphoma (MZL)
• Burkitt's lymphoma (BL)
• Post-transplant lymphoproliferative disorder (PTLD)
• Waldenström's macroglobulinemia / Lymphoplasmacytic lymphoma
• Other NK- or T-cell lymphomas (several types)

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.

References