Haematology - INCOMPLETE Flashcards
Question
Answer
Felty syndrome
Extra-articular manifestations of RhA = SANTA = autoimmune cytopenia + RhA Splenomegaly Arthritis Neutropenia Thrombopenia Anaemia
TTP
Deficiency of vWF cleaving protease (ADAMTS13)
Anti-ADAMTS13 antibody
↓protease –> ↑vWF multimers –> platelet thrombi form –> RBC fragmenttion
Platelets comsumed –> TTP
Tx: Plasma exchange
causes of reticulocytosis
Haemolysis, Haemorrhage, Haemolytic replacement
Causes of absent reticulosis
Inadequate haematoinics (iron, B12), BM failure, major haemorrhage
Dacrocyte
Tear drop RBC
DDx: Leuco-erythroblastic anaemia, Myelofibrosis
Echinocyte (= Burr cells)
Pyruvate kinase deficiency
Leuco-erythroblastic anaemia
Nucleated RBCs + Immature myeloid cells (myelocytes)
Dx Bone marrow infiltration (so both immature red and white cells spill out)
- Cancer
- Infection
- Myelofibrosis
Tx of Essential thrombocythaemia
Anagrelide
Target cells = Codocytes
IDA, Liver disease, Hyposplenism, Thalassaemia
Blood film of hyposplenism
Acanthocytes (many specules)
Howell-Jolly bodies
Target cells
Plummer vinson syndrome
IDA
Oesophageal webs
Dysphagia
Sideroblastic anaemia
Due to ineffective erythrooiesis - Alcohol excess, MDS Ring sideroblasts (ring of iron around nucleus in erythroid precursors)
Warm agglutinins
Autoimmune haemolytic anaemia (WAIHA) - more common \+ve Coomb's test Associated with : CLL, Lymphoma > 37oC IgG Spherocytes Tx: Steroids, Splenectomy
Cold agglutinin
Autoimmune haemolytic anaemia \+ve Coomb's test - Ab causes RBC to agglutinate and obstruct vessels when cold Mycoplasma infection, EBV infection < 37 oC IgM Avoid the cold
Hereditary spheocytosis
Spectrin deficiency
Autosomal dominant
↑osmotic fragility
Fanconi anaemia
Most common cause of inherited aplastic anaemia«
Mutation in DNA repair
Chromosomal fragility
Sx: aplastic anaemia + short stature + café au lait spots
βthalassaemia X-ray
Hairs on end of skull X-ray
Paroxysmal Cold Haemoglobinuria
Viral infection –> Hb in urine
Donath-Landsteiner antibodies –> stick to RBCs in cold –> complement activated on re-warming (self-limiting as IgG so dissociate at higher temp than IgM)
Paroxysmal Nocturnal haemoglobinuria
Loss of GPI on RBCs –> complement mediated lysis (esp at night)
Morning haemoglobinuria
Thrombosis
Ham’s test: in vitro acid induced lysis
TTP anitbody
Anti-ADAMTS13 antibody –> ↑VWF –> ↓Plt
Reverse Warfarin
Vitamin K
Prothrombin complex concentrate
Fresh frozen plasma
Stop warfarin
Scoring system for DVT/PE
Wells score
Unfractionated heparin - monitoring
Monitor APTT
Antidote for heparin
Protamine sulphate
S/E of Heparin
Bleeding
Heparin-induced thrombocytopaenia
Target INR for 1st DVT or AF
2.0 - 2.5
Target INR for recurrent DVT
2.5 - 3.0
Target INR for AF
2..0 - 3.0
Target INR for mechanical prosthetic valve
2.5 - 3.5
CLL staging
Rai-Binet staging
Lymphoma staging
Ann-Arbor staging
MM staging
Durie-Salmon staging system
Dyskeratosis congenita
Telomere shortening
Triad of skin pigmentation, nail dystrophy, oral leukoplakia + BM failure
Schwachman-Diamond syndrome
Neutrophilia
Skeletal abnormalities
Diamond-Blackfan syndrome
Pure red cell aplasia, normal WCC, normal Plt
Dysmorphic
Acute transfusion reaction
ABO Febrile non-haemolytic (no change in BP) Anaphylaxis TRALI TACO Bacterial infection
Delayed transfusion reaction
Delayed haemolytic transfusion - rhesus
Post-transfusion purpura
GVHD
Viral infection
Cut-off between MGUS and MM
Monoclonal protein < 30
BM plasma cells < 10%
Smouldering MM
> 30g/L M spike (paraprotein)
10% clonal plasma cells
No CRAB (i.e. no end organ damage)
Non-secretory myeloma
MM without M protein in blood or urine
Solitary plasmacytoma
Bone lytic lesions (B of CRAB) but no other findings of MM
