Hodgkins lymphoma: unlike other lymphomas – has a bimodal age distribution (15-35, >55yo). Hodgkins is characterized by the spread of disease from one lymph node group to another and by the development of systemic symptoms with advanced disease. Histopath typically shows multinucleated Reed-Sternberg cells. Staging involves thorough imaging (CT, MRI, PET – plus biopsy) and is by the Ann Arbor staging clasification. Tx is with RT, CT (ABVD, Stanford V or BEACOPP regimens), and stem cell transplantation. Risk factors for HL = infectious mononucleosis (caused by EBV), male gender, HIV/immunocompromised. Prognosis is not known, but if mild grade >90% survive >10years.
Non-Hodgkins Lymphoma (NHL): A term used to describe all lymphomas except Hodgkins.
Other chronic myeloid disorders:
Myeloproliferative diseases =
Myelofibrosis: isa myeloproliferative disease in which the proliferation of an abnormal type of bone marrow stem cell results in fibrosis, or the replacement of the marrow with collagenous connective tissue fibers. Symptoms/signs include: HSM, bone pain, thrombocytopaenia, fatigue, neutropaenia, anaemia, hyperuricemia and gout (secondary to high cell turnover). Eventual and progressive pancytopaenia. Tx: The only known cure is allogeneic stem cell transplantation. Other supportive treatment options include folic acid, allopurinol, blood transfusions, dexamethasone,alpha-interferon, hydroxycarbamide, lenalidomide and thalidomide.
Polycythemia Rubra Vera: is a disease state in which the proportion of blood volume that is occupied by red blood cells (haematocrit) increases. In PRV abnornmal RBC are produced in BM causing elevated haematocrit (Hct > 55% seen in 83% of cases). Symptoms/signs = headaches, vertigo, HSM, HTN, thromboses. Tx blood letting.
- Cause: mutations in JAK2 are found in 95% of cases.
- Mutations in JAK2 have also been implicated in essential thrombocythemia, and other myeloproliferative disorders
- This mutation is a change of valine to phenylalanine at the 617 position, whcih appears to render hematopoietic cells more sensitive to growth factors such as erythropoietin and thrombopoietin.
Essential / primary thrombocythaemia (also called thrombocytosis): a high platelet count (Plt >750) which can lead to thromboses in some patients. Tx: often not indicated. Low dose aspirin or hydroxyurea can improve symptoms.