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.)
List of diseases
The hematological malignancies include:
Related disorders, which are generally not called "cancer":
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.
v • d • e Pathology: hematology (primarily D50-D77, 280-289) |
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RBCs/anemia/ hemoglobinopathy (Myeloid) | nutritional anemia: Iron deficiency anemia, Plummer-Vinson syndrome, Megaloblastic anemia (Pernicious anemia) hereditary hemolytic anemia: G6PD Deficiency, Thalassemia, Sickle-cell disease/trait, Hereditary spherocytosis, Hereditary elliptocytosis, Hereditary stomatocytosis
acquired hemolytic anemia: Autoimmune (Warm), HUS, MAHA, PNH, PCH
aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia • Hemochromatosis |
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Coagulation/platelets (Myeloid) | coagulopathy: DIC (Congenital afibrinogenemia, Purpura fulminans) • Hemophilia (A/VII, B/IX, C/XI, XIII) • Von Willebrand disease
Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP
primary hypercoagulable state: Protein C deficiency -
Protein S deficiency - Antithrombin III deficiency - Factor V Leiden - Activated protein C resistance - Antiphospholipid syndrome
other hemorrhagic conditions: Bernard-Soulier syndrome - Glanzmann\'s thrombasthenia - Grey platelet syndrome - May Hegglin anomaly
Essential thrombocytosis - Thrombocytopenia |
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Monocytes/Macrophages (Myeloid) | WHO-I histiocytosis (Langerhans cell histiocytosis)
WHO-II/non-Langerhans-cell histiocytosis (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis)
WHO-III/malignant histiocytic disorders (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease)
-cytosis: Monocytosis
-penia: Monocytopenia |
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Granulocytes (Myeloid) | -cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia) -penia: Granulocytopenia/agranulocytosis (Neutropenia, Kostmann syndrome) |
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| Other/general myeloid | Chronic granulomatous disease |
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| Lymphoid | -cytosis: Lymphocytosis -penia: Lymphopenia |
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| Other | Asplenia/hyposplenism - Methemoglobinemia - Pancytopenia |
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| See also hematological malignancy and immune disorders |
v • d • e Hematological malignancy histology (ICD-O 9590-9989) |
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| Lymphomas (9590-9759) | Hodgkin\'s lymphoma vs. Non-Hodgkin lymphoma - Diffuse lymphoma vs. Follicular lymphoma
B-cell lymphoma (Small cell, Primary effusion, Diffuse large, ,Burkitt\'s, Splenic marginal zone, MALT)
T-cell lymphoma (Cutaneous , Mycosis fungoides/Sézary\'s disease, Angioimmunoblastic, Anaplastic large cell, Hepatosplenic)
plasma cell (Plasmacytoma, Multiple myeloma)
mast cell tumor (Mast-cell sarcoma, Malignant mastocytosis, Malignant histiocytosis, Langerhans cell histiocytosis) |
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| Immunoproliferative disorders (9760-9799) | Waldenström macroglobulinemia - Lymphomatoid granulomatosis |
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| Lymphoid leukemias (9800-9839) | ALL - CLL - T-cell leukemia (Adult, Large granular lymphocyte, Prolymphocytic, Acute lymphoblastic) - B-cell leukemia (Prolymphocytic) |
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| Myeloid leukemias (9840-9939, 9963) | AML (M2, APL/M3, AMoL/M5, Erythroleukemia/M6, AMKL/M7) - CML (CMoL, CNL, Philadelphia chromosome) - Granulocytic sarcoma |
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| Other leukemias (9940-9949) | Hairy cell leukemia - Aggressive NK-cell leukemia |
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| Myeloproliferative disease (9950-9961) | Polycythemia vera - Essential thrombocytosis - Myelofibrosis |
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| Other (9964-9989) | Hypereosinophilic syndrome - Post-transplant lymphoproliferative disorder - Myelodysplastic syndrome |
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| See also hematology and immune disorders |
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