307. Non hodgkin's lymphoma, myeloproliferative disorders and complications of leeakemia Flashcards
What is a non hodgkins lymphoma?
Lymphoma that doesnt contain reed stenburg cells
What are the causes of non-hodgkins lymphoma?
Immunodeficiency
Drugs
HIV
H pylori
Toxins
What are the signs of non hodgkins lymphoma?
Superficial lymphadenopathy
Extranodal disease
Systemic symptoms- fevers, night sweats
Pancytopenia
What are common extra-nodal sites in non-hodgkins lymphoma?
Gut- commenest. Gastric MALT (h. pylori), Non malt gastric, small bowel
Skin
Ororpharynx
Bone, CNS and lung
What blood tests can be done for Non hodgkins lymphoma?
FBC, U&E’s, LFT’s increasde LDH
Marrow and node biopsy
Send cytology of any effusion
What is prognosis for Non hodgkins lymphoma?
Low grade lymphomas- Often causes little symptoms, hard to cure. e.g. follicular and MALT
High grade lymphomas- often curable, e.g. burkett’s
What is the treatment for non hodgkins lymphoma?
Low grade- radiotherapy, interferon alpha and rituximab
High grade- (b cell)- R- rituximab C-yclophasmide H-ydroxydaunorubicin O-ncovin P-rednisolone
Where are blood cells produced
What is meant by extramedullary haematopoiesis
Central skeleton and proximal long bones
In some anaemias (thalaseammia) increasde demand causes cells to made beyond the marrow in the liver and spleen.
What are the causes of pancytopenia?
Aplastic anaemia, acute leukaemia, myelodysplasia, myeloma, lymphoma, solid tumours, TB , megaloblastic aneamia, Hypersplenism
What is agranulocytosis
Implies that granulatocytes (neutrophils, basophil, eosinophil granules) have stopped being made
What drugs induce agranulocytosis
Carbimazole,
procainamide
sulfonamides
gold
clozapine
dapsone
How do you carry out a bone marrow biopsy
Aspirate and trephine (fluid and bone marrow)
taken from posterior ilaic crest
What is aplastic anaemia?
Bone marrow stops making cells- pancytopeania
Presents with signs of anaemia
What are the causes of aplastic anaemia?
Autoimmune
Drugs
Viruses (parovirus and hepatitis)
Irradiation
What is the treatment of aplastic anaemia?
Supportive- transfuse blood products
Allogenic blood transfusion
Immunossuppresion
What are myeloproliferative disorders?
Proliferation of haemtopietic myeloid stem cells in the bone marrow
Polycythaemia vera- RBC
Chronic myeloid leukemia- CML
Essential thrombocytopeania
FIbroblasts- Myelofibrosis
What is reactive polycythaemia?
Increased RBC, due to reduced plasma volume
Can be acute due to dehydration or chronic- obesity, hypertension and high alcohol intake
What is absolute polycythaemia?
Increased RBC mass can be primary- polycythaemia vera
or secondary due to high altitude, chronic lung disease, cyanotic heart disease, heavy smoking, increased EPO secretion
What is polycythaemia vera?
Malignant proliferation of plueripotent cells leading to them being resistant to apoptosis and causing excess RBC, WBC and platelets
How does polycythaemia vera present?
Asymptomatic
Headaches, tinnitus, visual distrubance
Itching after hotbath, Burning sensation in finger and toes
How is PCV investigated?
FBC, reticulocyte, hb, wbc, latelets, b12
Marrow biopsy
Cytogenics to differntiate from CML
Serum erythropoitein
How is PCV treated
Keep heamatocrit <0.45 to reduce risk of thrombosis
Younger patients are given venesection
Hydroxycarbamide
How does essential thrombocytopeania present and how is it treated?
Bleeding
Arterial and venous thromboembolism
Headache, atypical chest pain
In essential thrombocytopeani it is important out causes of thrombocytosis. What are the causes of thrombocytosis
Bleeding
Infection
Chronic inflammation
Malignancy
Trauma
Iron deficiency
What is the presention of myelofibrosis
Night sweats, fever, weight loss
splenomegaly
bone marrow failure
What is the characterstic film seen in myelofibrosis
Leukoeryhtroblastic cells with tear drop RBC’s
How is myelofibrosis treated?
Bone marrow support
Allogenic stem cells