Os h1

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Os h1

  1. 1. OSCE PEDIATRICS HEMATOLOGY SPOTS PART 1 www.dnbpediatrics.com
  2. 2. Anatomy of PS www.dnbpediatrics.com
  3. 3. Red Cells • Circular • Mean cell diameter of 8 um(7.2-7.9) • Central pallor does not exceed more than one half the diameter of the cell. • Ideal part of PS– Red cells are just beginning to touch and overlap. www.dnbpediatrics.com
  4. 4. Platelets • Granular basophilic forms with a mean diameter of 1-2 um. • Platelet adequacy: - 8-15 platelets (individually or in small clumps) per 100x oil immersion field or - 1 platelet for every 10-30 RBCs. www.dnbpediatrics.com
  5. 5. Neutrophils • Round cells (10-14um) • 2-5 lobes connected by a thin thread of chromatin • Chromatin stains purple • Cytoplasm is pink with purple granules • Barr body www.dnbpediatrics.com
  6. 6. Band or Stab forms • Nucleus is U shaped • Rudimentary lobes connected by a thick band rather than a thread. www.dnbpediatrics.com
  7. 7. Eosinophil • Same size as neutrophils • 2 lobes • Nucleus lightly stained • Orange red granules all over the cell. www.dnbpediatrics.com
  8. 8. Basophil • Slightly smaller than neutrophils • Has deeply basophilic granules www.dnbpediatrics.com
  9. 9. Monocyte • Largest cells in peripheral blood • Nucleus– round, kidney-shaped,oval or lobulated and folded. • Cytoplasm is light blue or grey with fine purple granules www.dnbpediatrics.com
  10. 10. Lymphocyte • Small-10 um in diameter • Large-20 um in diameter • Nucleus-large rounded, densely stained • Cytoplasm-scanty, pale to dark blue. www.dnbpediatrics.com
  11. 11. N0RMAL SMEAR www.dnbpediatrics.com
  12. 12. An 8 month old female child BONCM brought with increasing pallor noticed since the last 1 month. • Diet h/o -- breast feeding with adequate food On examination, • Wellgrown • Pale and irritable • L-1.5 cm,S-1.5 cm. www.dnbpediatrics.com
  13. 13. www.dnbpediatrics.com
  14. 14. • 1) Describe peripheral smear • 2) Diagnosis on PS • 3) Give 4 causes of hypochromic microcytic • anemia • 4) give 2 PS changes which are not present that would point it to be a smear of thal trait. www.dnbpediatrics.com
  15. 15. • 1) hypo,micro, aniso, poikilo, tear drop cell • 2) hypo, micro anemia • 3) iron def • Thal • Sideroblastic anemia • Anemia of chronic disease • HbH • Atransferrinemia • 4) basophilic stippling and target cells. www.dnbpediatrics.com
  16. 16. After treatment www.dnbpediatrics.com
  17. 17. • 1) Describe PS • 2) Name 3 conditions in which dimorphic red cell population is seen. www.dnbpediatrics.com
  18. 18. • 1)Micro,hypo,macro, aniso,poikilo, tear drop cells, polychromasia • 2) iron def responding to iron therapy • After the transfusion of normal blood to a patient with a hypochromic anemia • 3) Sideroblastic anemia www.dnbpediatrics.com
  19. 19. • What would you do if the child with proved iron def after receiving adequate iron therapy comes back showing no response? www.dnbpediatrics.com
  20. 20. • Suspect poor compliance • Iron salt being used may be ineffective • Increased blood losses • GI causes • Worm infestations • H.Pylori • Giardiasis • Absorption defect • Coeliac disease • IBD • Autoimmune gastritis www.dnbpediatrics.com
  21. 21.  A 9 month male child has • fever since 15 days on and off • Pallor – 15 days • No other significant history  On examination, • Liver--2 cm • Spleen – 2.5cm • Rest of the examination -- Normal www.dnbpediatrics.com
  22. 22. www.dnbpediatrics.com
  23. 23. • 1) Describe the specific PS change which points it to being a homozygous Thal smear. • 2) Name 4 conditions with increased Hb F. • 3) Give the genotype of 4 transfusion independent thal intermedia conditions. www.dnbpediatrics.com
  24. 24. • 1) Nucleated red cells. • 2) increased HbF • HPFH • δβ thalassemia • Congenital red cell aplasia • Congenital aplastic anemia(Blackfan-Diamond and Fanconis anemia) • JCML • Some MDS www.dnbpediatrics.com
  25. 25. • Slight increase in erythropoeitic stress • (hemolysis, bleeding, recovery from acute BM failure, pregnancy). • 3) Genotype • Some β+/β+ thal homozygotes • Interaction of β0/ β0, β0/ β+ or β+/β+ with α thal. • Interaction of β0/ β or β+/β with triple α www.dnbpediatrics.com
  26. 26. • HbH disease • α 0 / Hb Constant Spring thal • Β0/δβ or Β+/δβ thal compound heterozygotes • δβ/ δβ thal • Some cases of HbE/β thal and Hb Lepore/ β thal • Rare cases of heterozygotes for β thal invol.exon 3 (dominant β thal) www.dnbpediatrics.com
  27. 27. A 10 yr old male child BONCM - Fever-low grade since 1 month - Easy fatiguability since 15-20 days -Tingling numbness in lower limbs since 10 days On examination- - Pallor ++ - Haemic murmur - Other systems--normal www.dnbpediatrics.com
  28. 28. www.dnbpediatrics.com
  29. 29. • 1) Describe PS • 2) Diagnosis on PS • Which other PS changes should have been present to support your diagnosis. • 3) Name at least 2 other conditions with neutrophilic hypersegmentation www.dnbpediatrics.com
  30. 30. • 1) macro,aniso,poikilo,hypersegmented neutrophils • 2)megaloblastic anaemia • Oval macrocytes,Baso stippling, HJ bodies,cabt rings • 3) hypersegmented neutrophils--- Uraemia • Iron def • Cytotoxic Rx esp. methotrexate • Hydroxycarbamide Rx www.dnbpediatrics.com
  31. 31. • 3) Define neutrophilic hypersegmentation • 4) Give 6 other differentials of macrocytosis www.dnbpediatrics.com
  32. 32. • 3) presence of 1 or more 6 lobed neutrophils or of 5 or more neutrophils with 5 or more well separated lobes among 100 segmented neutrophils. • 4) macrocytic anemia--- megaloblastic anemia • Aplastic anemia • Myelodysplastic syn. • Hydroxycarbamide Rx • Chronic liver disease • CDA type III • Benign familial macrocytosis • Hypothyroidism • Increased erythropoeisis. www.dnbpediatrics.com
  33. 33. • 2 weeks later,the child is clinically responding but the neutrophilic hypersegmentation is still persisting • Would you worry and change your line of Mx www.dnbpediatrics.com
  34. 34. 2 1 5 4 3 www.dnbpediatrics.com
  35. 35. • Name the circled cells and name the conditions in which they are seen www.dnbpediatrics.com
  36. 36. • 1) Pencil cells--- Iron def • 2) stomatocytes ---- hereditary stomatocytosis • Southeast Asian ovalocytosis • Liver disease • Alcoholism • Myelodysplastic syndromes • Artifact www.dnbpediatrics.com
  37. 37. • 3) Tear drop cell- Iron def anemia • Congenital hemolytic anemias • Microangiopathic hemolytic anemia • Oxidant damage • 4)Leptocyte---- iron def anemia • Thal • 5) Howell jolly body--- pernicious anemia • After splenectomy,splenic atrophy • Dyserythropoeitic anemia • Severe iron def www.dnbpediatrics.com
  38. 38.  A 6 yr old male child presents with fever of 2 days duration with acute abdominal pain since 1 day. He has never been symptomatic in the past.  On examination, • Pallor ++ • Icterus ++ • L3cm,S6cm • Other systems—normal. www.dnbpediatrics.com
  39. 39. CBC - Hb—9.8gm% -WBC—10,500/cumm - Hct—29.4% -P-55,L-45 - RBC—4 106 -Platelet—3.5 105 - MCV—85 fl - MCH---27 pg -Retic--- 5.6% - MCHC—34 % - RDW– 14.6% www.dnbpediatrics.com
  40. 40. www.dnbpediatrics.com
  41. 41. • 1) clinical diagnosis • 2) Name 4 conditions with spherocytosis • 3) Is Osmotic fragility test specific for hereditary spherocytosis. www.dnbpediatrics.com
  42. 42. • 1) Spherocytosis--- HS • ABO hemolytic disease • Autoimmune hemolytic anemia • Cl. Welchi sepsis • 2) NO www.dnbpediatrics.com
  43. 43. • 3) what is the mode of inheritance of HS • 4) Name 2 specific test to diagnose Hereditary spherocytosis. • 5) Give 5 causes of false positive DAT test www.dnbpediatrics.com
  44. 44. • 3) AD • 4) Cryohemolysis test • Memb. Protein analysis by Sodium dodecyl sulfate polyacrylamide gel electrophoresis • 5) alloantibody as in hemolytic disease of newborn or after an incompatible Tx • Drugs--- Quinidine,penicillin,cephalosporin • Sepsis • After ATG or ALG administration www.dnbpediatrics.com
  45. 45. • Gp O platelets with high titre antiAor B to gp A or B recipient. • IvIg may contain ABO or antiD antibodies. • As a result of antiphospholipid antibodies in APLA syndrome and SLE. • Sickle cell disease because of IgG coated red cells. www.dnbpediatrics.com
  46. 46. www.dnbpediatrics.com
  47. 47. • Sickling test on this patient is negative, explain • Which test next will you perform • Newborn screening ---- is it performed routinely and how? • If detected as sickle cell disease what steps will you take next? www.dnbpediatrics.com
  48. 48. • False negative • Low Hb • Outdated reagents • Infants younger than 6 months • Post Tx in which HbS level is < 20%. • False positive--- • Severe leucocytosis • Hyperproteinaemias • Unstable Hb esp. after splenectomy www.dnbpediatrics.com
  49. 49. • Newborn screening routinely performed in US and UK • Dried blood spot samples--- High Performance Liquid Chromatography or IsoElectricFocussing • Umbilical cord blood samples---- Hb electrophoresis using cellulose acetate or citrate agar or HPLC or IEF. • Parents CBC and Hb electrophoresis • Penicillin prophylaxis • Repeat babys Hb electrophoresis at 6 months of age. www.dnbpediatrics.com
  50. 50. • A 2.6 yr old asymptomatic child went for cough/cold. CBC showed microcytosis,put on hematinic. • On follow up, inspite of being treated adequately as a case of iron def anemia microcytosis persisted. • Referred to the hematologist. www.dnbpediatrics.com
  51. 51. www.dnbpediatrics.com
  52. 52. • 1) Name the cell • 2) Why do they occur • 3) 5 conditions in which target cells are seen www.dnbpediatrics.com
  53. 53. • 1) Target cell • 2)Disproportionately large surface compared to volume • 3) Liver disease • Hereditary hypobetalipoproteinemia • Iron def • Thal • Hbpathies--- HB C/β 0 thal • HbC disease www.dnbpediatrics.com
  54. 54. • Sickle cell anemia • Sickle cell/HbC disease • Sickle cell/ β thal • Hb E disease • Post splenectomy www.dnbpediatrics.com
  55. 55. www.dnbpediatrics.com
  56. 56. • 1) Name the intracellular inclusion • 2) Name the conditions in which it is seen. www.dnbpediatrics.com
  57. 57. • 1)Cabot ring • 2) lead poisoning • Pernicious anemia • Hemolytic anemias www.dnbpediatrics.com
  58. 58. www.dnbpediatrics.com
  59. 59. www.dnbpediatrics.com
  60. 60. • 1) Name the cell • 2) how will you investigate this patient www.dnbpediatrics.com
  61. 61. • 1) Basophilic stippling • 2) Megaloblastic anemia • lead poisoning • Thalassemia • Infections • Liver disease • Unstable hemoglbins • Pyrimidine nucleotidase 5 def www.dnbpediatrics.com
  62. 62. • A 2 yr old child has diarrhoea with oliguria with severe dehydration.The resident starts treatment and sends the CBC which shows low Hb with normal indices and low platelets. www.dnbpediatrics.com
  63. 63. www.dnbpediatrics.com
  64. 64. • 1)Name the cell • 2) Name 4 conditions in which these cells are seen • 3) Difference between acanthocyte and echinocyte • www.dnbpediatrics.com
  65. 65. • 1)Schistocytes and acanthocytes • 2) In certain genetic disorders( thal, CDA, hereditary pyropoikilocytosis) • Megaloblastic or dyserythropoeitic disorders • Microangiopathic hemolytic anemia/cardiac hemolytic anemias • Direct thermal injury www.dnbpediatrics.com
  66. 66. www.dnbpediatrics.com
  67. 67. • 3) Acanthocyte--- small no. of spicules of inconsistent length, thickness and shape, irregularly disposed over the surface of the cell. • Seen in abnormal phospholipid metabolism • Inherited abnormalities of RBC memb protein as in McLeod phenotype • Postsplenectomy • Hyposplenism • Liver disease www.dnbpediatrics.com
  68. 68. www.dnbpediatrics.com https://www.facebook.com/groups/dnbpediatrics/

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