Haematology Flashcards
Splenectomy
Causes: trauma, rupture, AIHA, ITP, HS, hypersplenism
Complications: Redistributive thrombocytosis (early VTE),
Gastric dilation, Left LL atelectasis, susception to infection (haemophilus, pneumo, menigo)
Mx: Immunisations (pnemococcal, HiB, Men C, Flu)
Daily Abx (Pen V or erythromycin)
Warning/alert card
Film: Howell-Jolly bodies, Pappenheimer bodies, target cells
Hyposplenism
Causes: • Splenectomy • SCD • Coeliac disease • IBD
Microcytic anaemia
Haem defect • IDA • Sideroblastic/lead poisoning • Anaemia of chronic disease Globin defect • Thalasaemia
Normocytic anaemia
- Recent blood loss
- Bone marrow failure
- Renal failure
- Early anaemia of chronic disease
- Pregnancy (Increased plasma volume)
Macrocytic anaemia
Megaloblastic • Vit B12 or folate def • Anti-folate drugs (phenytoin, methotrexate) • Cytotoxics Non-megaloblastic • Reticulocytosis • Alcohol or liver disease • Hypothyroidism • Myelodysplasia
Haemolytic anaemia
Acquired
• Immune mediate
o AIHA (warm, cold or PCH)
o Drugs (penicillin, quinine, methyldopa)
o Allo-immune (acute transfusion reaction)
• Paroxysmal nocturnal haemaglobinuria
• Mechanical
o MAHA (DIC, HUS, TTP)
o Heart valve
• Infection (malaria)
• Burns
Hereditary
• Enzyme (G6PD def and pyruvate kinase def)
• Membrane (Hereditary spherocytosis and eliptocytosis)
• Haemaglobinopathy (SCD and thalasthaemia)
Iron deficiency anaemia
Signs: koilonychias, angular stomatitis/glossitis/cheilosis, Plummer-Vinson syndrome.
Causes: Increased loss, decreased intake, malabsorption
Ix: Decreased ferritin, increased TIBC, decreased transferrin. Blood film (anisocytosis, poikilocytosis, pencil cells)
Imaging: Upper and lower endoscopy
Mx: ferrous sulphate 200mg PO TDS (SE: GI upset)
Sideroblastic anaemia
Ineffective erythropoiesis – red cells fail to form haem causes deposition of iron in mitochondria which rings around nucleus. Causes endo, liver and cardio damage
Congenital: delta-aminolevulinate synthase-2 def
Acquired: Myelodysplasia, alcohol, lead, anti-TB meds
Ix: Hypochromic microcytic anaemia, raised ferritin
BM biopsy: sideroblasts and increased iron stores
Mx: supportive + treat underlying cause
?pyridoxine
Hereditary spherocytosis
AD defect in RBC membrane, spherocytes get trapped in spleen – extravascular haemolysis
Features: splenomegaly, jaundice, pigmented gallstones
Complications: Aplastic crisis, megaloblastic crisis
Ix: ↑osmotic fragility, spherocytes, DAT -ve
Mx: folate and splenectomy
G6PD deficiency
X-linked disorder of pentose phosphate shunt, ↓NADPH production – RBC oxidative damage
Mediterranean and far east populations
Haemolysis triggers: fava bean, naphthalene (moth balls), infection, drugs (antimalarias, henna, dapsone, sulphonamides)
Ix: Film (irregularly contracted cells, Bite cells, ghost cells, blister cells, Heinz bodies) G6PD assay
Mx: treat infection, stop precipitant, transfusions
Coagulopathy
Vascular disorders
Vascular disorders • Congenital o HHT o Ehler’s Danlos syndrome o Pseudoxantoma elasticum • Acquired o Senile purpura o Vit C def o Infection (meningococcus) o Steroids o Vasculitis
Coagulopathy
Platelet disorders
Thrombocytopenia • Decreased production o BM failure (aplastic, inflitraion, drugs) o Megaloblastic anaemia • Increased destruction o Immune: ITP, SLE, CLL, heparin, viruses o Non-immune: DIC, TTP, HUS, PNH, APLS • Splenic pooling o Portal HTN o SCD Functional defects Drugs (aspirin, clopidogrel) Secondary (paraproteinaemias, uraemia) Hereditary • Bernard-Soulier GpIb def • Glanzmann’s: GpIIb/IIIa def
Coagulopathy
Coagulation disorders
Acquired: • Severe liver disease • Anticoagulants • Vit K def Congenital: • Haemophilia A: F8 def • Haemophilia B: F9 def • VWD
Immediate blood transfusion reactions
Immediate: • Haemolytic • Bacterial contamination • Febrile non-haemolytic • Allergic • TRALI • Fluid overload • Massive transfusion
Delayed blood transfusion reactions
Delayed: • Delayed haemolytic • Iron overload • Post-transfusion purpura • GvHD
Aplastic anaemia
Rare stem cell disorder, 15-24 yrs and >60yrs
Features: pancytopenia, hypocellular marrow
Inherited:
• Fanconi anaemia (Ashkenazi, short pigmented)
• Dyskeratosis congenital (premature ageing)
• Swachman-Diamond syn (+ exocrine pancreatic failure)
Acquired:
• Drugs
• Viruses (parvivirus, hepatitis)
• Autoimmune (SLE
Ix: BM biopsy
Mx: supportive – transfusion
Immunosuppression
Allogenic BMT (may be curative)
Bone Marrow failure causes
Congenital: Fanconi anaemia Acquired: • Idiopathic aplastic anaemia • BM infiltration • Haematological (Leukaemia, lymphoma, myelofibrosis, myelodysplasia, megaloblastic anaemia) • Infection: HIV • Radiation • Drugs: Cytotoxic, Abx (chloramphenicol, sulphonamides), diuretics (thiazides), anti-thyroid (carbimazole), Anti-psychotic (clozapine), Anti-epileptic (phenytoin)
Drugs causing bone marrow failure
Cytotoxic (cyclophosphamide, azathioprine, methotrexate) Abx (chloramphenicol, sulphonamides) diuretics (thiazides) Anti-thyroid (carbimazole) Anti-psychotic (clozapine) Anti-epileptic (phenytoin)
Myeloproliferative conditions
- Polycythaemia vera
- Essential thrombocythaemia
- Primary myelofibrosis
- CML
Acute lymphoblastic leukaemia
Children 2-5 yrs, rare in adults (worse prognosis)
Arrested lymphoblast development and proliferation
80% B lineage, 20% T lineage
RF: Genetic, radiation, Down’s
Features: BM failure, lymphadenopathy, orchidomegaly, thymic enlargement, hepatosplenomegaly, bone pain
CNS: palsies, meningism
Ix: ↑WCC↓RBC,PMN, plats. Bone marrow aspirate
>20% blasts
Mx: supportive (blood productions, allopurinol), Infection prophylaxis.
Chemo: 1. Remission 2. Consolidation 3. Maintenance
BMT – best option for younger patients
Chronic lymphocytic leukaemia
Features: asymptomatic, anaorexia, weight loss, bleeding, infections, lymphadenopathy
Complications: hypogammaglobulinaemia (causing infections), warm AIHA, Richter’s transformation
Ix: film – smudge cells, immunophenotyping
Chronic myeloid leukaemia
Philadelphia chromosome t(9;22) BCR-ABL gene
Features: middle age, anaemia, weight loss, abdo pain, splenomegaly
Mx: Imatinib (Inhibits tyrosine kinase associated with BCR-ABL defect), hydroxyurea, Interferon-alpha, allogenic BMT
Hodgkin’s lymphoma
Malignancy proliferation of lymphocytes with Reed-Sternberg cells. Bimodal age distribution (3rd and 7th decades)
Types:
1. Modular sclerosing
2. Mixed cellularity
3. Lymphocyte predominant
4. Lymphocyte depleted
Features: Weight loss, fever, night sweats
Non-Hodgkin’s lymphoma
Burkitts lymphoma t(8;14)
Mantle cell lymphoma t(11;14)
