bone marrow failure, myeloproliferative and myelodysplastic syndrome Flashcards
what can cause pancytopenia?
decreased marrow production
- aplasic anaemia
- infiltration e.g acute luekaemia, myeloma, lymphoma etc)
- megaloblastic anaemia
- myelofibrosis
Increased peripheral destruction
- hypersplenism
what is agranulocytosis and what drugs can cause it?
cessation of granulocyte production (neutrophils, basophils, eosinophils - BEN) leaving the patient at risk of fatal infection
- carbimazole
- procainamide
- sulfonamides
what are the S&S of aplastic anaemia?
Hx of recurrent infection fatigue Pallor Hx or bleeding or bruising Tachycardia and dyspnoea
what would blood analysis and bone marrow biopsy show in aplastic anaemia?
pancytopenia
low reticulocytes
bone marrow biopsy shows hypocellular marrow with no abnormal cells
what mutations are present in myeloproliferative disorders?
JAK2 mutations
what is polycythaemia rubra vera?
clonal haematopoietic disorder charcterised by erythrocytosis and often also involving thrombocytosis, leucocytosis and splenomegaly
what are the S&S of PRV?
hyperviscosity related symptoms i.e headaches, dizziness, tinnitus and visual disturbances
itching after hot bath
burning sensation in fingers and toes
Features of thrombosis - stroke etc
facial plethora and splenomegaly
Gout due to increased urate from RBC turnover
what would inv show in PRV?
Increased RCC, Increased Hb, Increased PCV, often increased WCC and platelets
Increased B12 and decreased serum EPO
Hypercellularity on bone marrow biopsy
what is essential thrombocytosis?
chronic myeloproliferative disorder associated with sustained megakarocyte proliferation with increased circulating platelets and associated with thrombosis and bleeding
what are the S&S of ET?
splenomegaly
microvascular occlusion - headache, chest pain, light headedness
Arterial and venous thrombosis
what does investigations for ET show?
increased platelets
peripheral blood smear = immature precursors and large platelets
what are the treatments for aplastic anaemia?
immunosupression - antithymocyte globulin, methylprednisolone, ciclosporin
Blood product transfusions
Abx and antifungals
Stem cell transplant
what is myelodysplastic syndrome?
a group of clonal stem cell disorders characterised by ineffective and dysplastic haematopoiesis resulting in 1 or more cytopenias and predilection to developing AML
Blast cells <20%
what are the signs and symptoms of MDS?
typically asymptomatic
anaemia symptoms
thrombocytopenia symtoms (petechiae and purpura)
bacterial infections due to neutropenia
what do investigations show in MDS?
FBC - cytopenias
Reticulocyte count = inappropiately normal in anaemia
Bone marrow aspirate = dysplasia and blasts <20%
BM biopsy = hypercellular marrow
