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Os h1 Os h1 Presentation Transcript

  • OSCE PEDIATRICS HEMATOLOGY SPOTS PART 1 www.dnbpediatrics.com
  • Anatomy of PS www.dnbpediatrics.com
  • 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
  • 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
  • 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
  • Band or Stab forms • Nucleus is U shaped • Rudimentary lobes connected by a thick band rather than a thread. www.dnbpediatrics.com
  • Eosinophil • Same size as neutrophils • 2 lobes • Nucleus lightly stained • Orange red granules all over the cell. www.dnbpediatrics.com
  • Basophil • Slightly smaller than neutrophils • Has deeply basophilic granules www.dnbpediatrics.com
  • 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
  • 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
  • N0RMAL SMEAR www.dnbpediatrics.com
  • 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
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  • • 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
  • • 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
  • After treatment www.dnbpediatrics.com
  • • 1) Describe PS • 2) Name 3 conditions in which dimorphic red cell population is seen. www.dnbpediatrics.com
  • • 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
  • • What would you do if the child with proved iron def after receiving adequate iron therapy comes back showing no response? www.dnbpediatrics.com
  • • 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
  •  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
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  • • 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
  • • 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
  • • 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
  • • 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
  • 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
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  • • 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
  • • 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
  • • 3) Define neutrophilic hypersegmentation • 4) Give 6 other differentials of macrocytosis www.dnbpediatrics.com
  • • 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
  • • 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
  • 2 1 5 4 3 www.dnbpediatrics.com
  • • Name the circled cells and name the conditions in which they are seen www.dnbpediatrics.com
  • • 1) Pencil cells--- Iron def • 2) stomatocytes ---- hereditary stomatocytosis • Southeast Asian ovalocytosis • Liver disease • Alcoholism • Myelodysplastic syndromes • Artifact www.dnbpediatrics.com
  • • 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
  •  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
  • 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
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  • • 1) clinical diagnosis • 2) Name 4 conditions with spherocytosis • 3) Is Osmotic fragility test specific for hereditary spherocytosis. www.dnbpediatrics.com
  • • 1) Spherocytosis--- HS • ABO hemolytic disease • Autoimmune hemolytic anemia • Cl. Welchi sepsis • 2) NO www.dnbpediatrics.com
  • • 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
  • • 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
  • • 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
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  • • 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
  • • 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
  • • 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
  • • 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
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  • • 1) Name the cell • 2) Why do they occur • 3) 5 conditions in which target cells are seen www.dnbpediatrics.com
  • • 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
  • • Sickle cell anemia • Sickle cell/HbC disease • Sickle cell/ β thal • Hb E disease • Post splenectomy www.dnbpediatrics.com
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  • • 1) Name the intracellular inclusion • 2) Name the conditions in which it is seen. www.dnbpediatrics.com
  • • 1)Cabot ring • 2) lead poisoning • Pernicious anemia • Hemolytic anemias www.dnbpediatrics.com
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  • • 1) Name the cell • 2) how will you investigate this patient www.dnbpediatrics.com
  • • 1) Basophilic stippling • 2) Megaloblastic anemia • lead poisoning • Thalassemia • Infections • Liver disease • Unstable hemoglbins • Pyrimidine nucleotidase 5 def www.dnbpediatrics.com
  • • 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
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  • • 1)Name the cell • 2) Name 4 conditions in which these cells are seen • 3) Difference between acanthocyte and echinocyte • www.dnbpediatrics.com
  • • 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
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  • • 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
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