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Haematology

Haematology. Dr Dániel Erdélyi 2nd Dept Pediatrics Semmelweis University. Haematology topics for final exam. Coagulopathies Leukaemia Hodgkin’s disease, Non-Hodgkin’s lymphoma Diseases with hepatomegaly, splenomegaly Lymphnodes enlargement - DD and treatment Anaemias

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Haematology

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  1. Haematology Dr Dániel Erdélyi 2nd Dept Pediatrics Semmelweis University

  2. Haematology topics for final exam • Coagulopathies • Leukaemia • Hodgkin’s disease, Non-Hodgkin’s lymphoma • Diseases with hepatomegaly, splenomegaly • Lymphnodes enlargement - DD and treatment • Anaemias • Iron deficiency anaemia

  3. 1st case presentation 6 mo. old boy C/O ↑crying, progressively over recent days. O/E well developed and hydrated, CVS stable, Abdo distended, liver 1.5–2 cms below costal margin, Exam unremarkable otherwise WBC 14.3 G/L, Neutro 13%, Lymph 76% Hb 102 g/L, Plt 379 G/L Creat 25 umol/L, U&Es-CRP normal Ideas, please! Lab values normal for adults would be very worrying in a baby!

  4. 1. lab normal ranges

  5. Normal Hb in infancy & childhood Hb (g/L) Hb (g/L) 200 200 boys girls 150 100 100 Age Age 3 mo 10 y 6 mo 3 mo 6 mo

  6. 3mo - 2 years < 100 < 110 6 - 14 years < 120 Adult women < 120 Adult men < 130 Anaemia, thresholds Age Hemoglobin (g/l) 2 - 5 years

  7. Normal WBC in infancy & childhood WBC (G/L) 30 15 10 5 Age 10 y 3 mo 6 mo

  8. Differential count in children 1 wk 4 y 10 y Neutrophils Lymphocytes

  9. Normal platelets in childhood Plt (G/L) 300 150 Age

  10. „Adult type” FBC in 6 mo baby(case presentation – to interpret) • Hb 140 g/l • WBC 4.5 G/L • Neutrophils 70%

  11. 2nd case presentation 18 mo. girl Pale, GP requested FBC WBC 12.5 G/L, Neutro 26%, Hb 82 g/L, MCV 59 fl, RDW 18%, Plt 530 G/L, Physical exam unremarkable Interpretation, DD, please!

  12. Classification of anaemia 1. Blood loss • acute • chronic – iron deficiency later • external • internal

  13. 2A. Haemolytic - intrinsic • membrane-defects (sphaerocytosis, elliptocytosis, stomatocytosis, PNH) • hemoglobinopathy (sickle cell anaemia, thalassaemia, rare others) • enzyme-defects (pyruvate kinase defect, glucose-6-phosphate dehydrogenase def., other rare forms)

  14. 2B. Haemolytic – extrinsic Immune Autoimmune haemolytic anaemia Alloimmune (neonatal) Autoimmune diseases Transfusion reactions Non-imm. Infections (malaria, mycoplasma, EBV) +/- immune mechanisms Hypersplenism Microangiopathic (HUS, TTP, DIC, Kassabach-Merritt syndrome) Not typically called as haemolytic anaemia

  15. 3. Hyporegenerative • Haematinics: Fe, Cu, folate, B6, B12, protein... • Primary or idiopathic: congenital anaemias, SAA • Temporary, by external causes: viral infections (Parvo B19, EBV, CMV, else) drugs (ibuprofen, metamizol; chemotherapy) • Bone marrow space ↓ leukaemia, neuroblastoma, osteopetrosis... • Secondary other: chronic infections, renal, hepatic, endocrine disorders autoimmune (SLE)

  16. 2. DIAGNOSTICS

  17. Microcytic Normocytic Macrocytic Iron deficiency Blood loss B12 folate deficiency Chronic disease Haemolysis/AIHA Liver disease Thalassaemia Bone marrow Aplastic failure MDS Lead intox. Renal failure Congen dyserythr. a B6 deficiency Hypothyroidosis Gravidity Various: corpuscular haemolytic anaemias

  18. RDW: red cell distribution width • Normal: 11-14% • Increased: iron deficiency, cong. membrane disorders, burns, microangiopathic Hypochrome / Hyperchrome • Usually correlate with micro/normo/macrocytic featire • Sphaerocytosis: ↓ MCV ↑ MCHC

  19. Reticulocyte count • Normal: 0.5 – 1.5 %, but consider anaemia • High: bleed, haemolysis • Low: hyporegenerative Other lab tests to identify haemolysis: • Billirubin • LDH, haptoglobin • Consider intra/extravascular haemolysis

  20. Blood film Iron deficiency

  21. Thalassaemia

  22. Sphaerocytosis

  23. Elliptocytosis

  24. Megaloblastic anaemia

  25. Fragmentocytes

  26. Sickle cell disease

  27. Lead intoxication

  28. Bone marrow cytology/histopath. • For hyporegenerative anaemias only Antibodies • For haemolytic anaemias only • Coombs: direct (DAT) / indirect antibodies • Cold and warm antibodies • Specific antibodies

  29. Miscellaneous others • Haemoglobin electrophoresis / HPLC • Osmotic fragility / cryoresistance • ADAMTS13 activity • D-dimer, coagulation factors • Haemoglobinuria • Erythropoietin

  30. 3. CLINICAL COURSE @ THERAPY

  31. Iron deficiency anaemia • Prelatent / latent deficiency / anaemia • Causes • Symptoms: haematology, others • Lab signs; DD chronic disease related a. • Therapy • iron salts (ferrous sulphate II, III) • iron carbohydrate complex • iv iron • Follow up

  32. Haemolytic anaemias Clinical signs: • Pallor, collapse • Icterus • Splenomegaly • Gall stones • Possibly emergencies: rapid haemolysis, aplastic crisis

  33. Immune-haemolytic anaemia • Course of AIHA • Therapy: • glucocorticoids • IVIG • other immunosuppressive therapies, rituximab • splenectomy • transfusion only when life-threatening

  34. Haemoglobinopathies www.wikipedia.org

  35. Sickle cell anaemia – special points • Painful cryses • Sequestration • Hydration • Analgaesia • Hyposplenism • Transfusion policy • Exchange transfusion • Transcranial Doppler-US • Hydroxyurea, allo-HSCT

  36. Congenital aplastic/hypoplastic anaemias • Fanconi anemia • Dyskeratosis congenita • Shwachmann-Diamond sy • Constitucional aplastic anemia • Diamond-Blackfan anaemia • ...

  37. Fanconi anaemia • DNA repair defect • Mean age at diagnosis 9 years, pancytopenia • Multiple anomalies possible: short stature, bone, kidney, heart, malformations, skin pigmentation, hypogonadism, learning difficulties

  38. Diamond-Blackfan anaemia • Ribosomal defect • Isolated red cell aplasia, presents at few months of age • Most children without other abnormalities

  39. Treatment of congenital aplastic / hypoplastic anaemias • Symptomatic: transfusions, kelating agents • Steroids • Allogenic stem cell transplant

  40. 4. learning points

  41. Frequent, important... Anaemia - most important: • Iron deficiency: dietary, cow-milk protein allergy, malabsorption • Anaemia in chronic diseases 2nd line • B12 and folate deficiency • Corpuscular (e.g. sphaerocytosis) and autoimmune haemolytic anaemias • In Hungary, haemoglobinopathies are rare

  42. Bleeding - most important: • ITP, DD of thrombocytopenia • Haemophylia A, B • von Willebrand’s 2nd line • DIC, HUS • Lupus anticoagulans and other inhibitors

  43. Haemat. malignancies – most important: • ALL >> AML >> CML • Hodgkin’s • Non-Hodgkin: • Burkitt’s • Lymphoblastic lymphoma

  44. Specific to / different in children Alloimmune cytopenias in newborns + thrombocytopenia from maternal ITP Vitamin K deficiency in infants Presenting symptoms of congen. coagulopathies Frequency of malignancies Approach to polycytaemia, thrombocytosis

  45. 5. ELSE

  46. New leukaemic patient • New malignancy • Febrile neutropenia

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