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APPROACH TO ANEMIA
DR. V. B. KASYAPA. J
1st year GM PG
Date : 05/08/16
ERYTHROPOESIS
Erythroid/Megakaryocytic
Progenitor
Erythroid Cells
Pronormoblast
RBC
GATA-1 & FOG-1
EPO
4-5 DIVISIONS
WHO Definition :
Anaemia is a condition in which the
number of red blood cells (and
consequently their oxygen-carrying
cap...
Clinical Key
• Classical symptoms :
– Fatigue, Malaise
– Dyspnoea on exertion
– Loss of stamina
– Palpitations
– Complaint...
Clinical Key
• Symptoms suggestive of Nutritional anemia :
– Blood in stools
– Loose stools/ Clay colour stools
– Constipa...
Clinical Key
• Symptoms of Haemolysis :
– Yellowish discoloration of eyes and skin
– Reversible skin pigmentation
• Sympto...
Clinical Key
• Symptoms suggestive of genetic disorders :
– Early hair greying
– Short stature
– Dystrophic nails
• Past H...
Clinical Key
• O/e :
– Mental state
– Signs of dehydration
– Cheilosis (Fissures at corners of mouth)
– Koilonychia (Spoon...
Clinical Key
Clinical Key
– Icterus
– Clubbing
– Cyanosis
– Lymphdenopathy
– Pedal edema
– Petechiae/ Ecchymosis
• Systemic Examination...
Clinical Key
• Also Look for :
– Ascites
– Heart failure
– Cirrhosis
– Endocrinopathies
– Pseudoxanthoma elasticum
– Neopl...
Lab Investigations
• Complete Blood counts
– Hb :
Occasion Normal Value (mg/dL)
At Birth 17
Childhood 12
Adolescence 13
Ad...
Lab Investigations
– Hematocrit :
– Reticulocyte Count
– Absolute reticulocyte Count
– Recticulocyte Production Index
Norm...
• RBC Indices
• Other Counts
– TLC
– DLC
– Nuclear Segmentation of Neutrophils
– Platelet Count
Lab Investigations
Indices...
• Red cell Morphology
– Cell size
– Mirocytic (<80 fL)
– Macrocytic (>100 fL)
– Anisocytosis ( Variable size )
– Poikilocy...
• Iron Studies
Lab Investigations
Study Normal Range
Serum Iron 50-150 µg/dL
TIBC 300-360 µg/dL
Serum Ferritin Males – 100...
• Marrow Examination
– Aspirate :
– M/E ratio (1:1)
– Cell Morphology
– Iron Stain
– Biopsy
– Cellularity (1:1)
– Morpholo...
Lab Investigation
Lab Investigation
Clinical Classification of Anaemia
Reticulocyte Count
• By Supra vital stains – Blue, black punctate
spots (precipitated rRNA)
• Life – 24 to 36 hrs
• Normal...
Reticulocyte count
Reticulocyte count
• Correction for Anemia
– 𝐴𝑏𝑠𝑜𝑙𝑢𝑡𝑒 𝑅𝑒𝑡𝑖𝑐𝑢𝑙𝑜𝑐𝑦𝑡𝑒 𝑐𝑜𝑢𝑛𝑡 =
𝑟𝑒𝑡𝑖𝑐𝑢𝑙𝑜𝑐𝑦𝑡𝑒 𝑐𝑜𝑢𝑛𝑡 𝑥 𝐻𝑏/15 𝑜𝑟 𝐻𝑚𝑡/45
• Correcti...
Clinical Classification of Anaemia
Hypo-proliferative Anemia
• Causes :
– Mild to Moderate Anemia
– Marrow Damage
– Inadequate EPO stimulation
• Inflammation...
Hypoproliferative Anemia
Parameter Mild – Mod Iron Def Chr. Inflammation
S.Iron ↓ ↓
TIBC ↑↑ ↓/N
% Saturation ↓ ↓
S.Ferriti...
Maturation Disorder
• BM shows erythroid hyperplasia (M/E < 1:1)
– But fails to release into peripheral circulation
Nuclea...
Blood Loss
• Normocytic/ Slightly Macrocytic
Acute Blood
Loss
• No Increase in RPI
• Signs of
Hypovolemia
dominate
Sub Acu...
Intra Vascular
(Iron loss may limit the
Marrow response)
Acute
(Autoimmune hemolysis/
Pathway defects )
Extra Vascular
(Ef...
Iron Deficiency Anemia
• C/f :
– Pregnancy/ Adolescence/ Blood Loss/ Phlebotomy
– Advanced tissue iron def
» Cheilosis
» K...
Iron Deficiency Anemia
• RBC Indices
– Microcytic, Hypochromic, Aniso-Poikilocytosis
• Iron Studies
• Marrow Iron (Normal ...
Iron Deficiency Anemia
DD for Microcytic Anemia
Iron Deficiency Anemia
Red cell infusion
(Symptomatic/ CV instability/
Elder/ Continued blood loss)
Oral Iron
(Young/ Asym...
• Oral Iron:
– 200mg elemental Iron/day
– Absorption will be upto 50mg/d (with good retention
capacity)
– Supports 2-3x pr...
– Iron Tolerance test
• 2 Iron tabs on empty stomach
• Serial S.Iron for 2 hrs
• At least 100 mcg/dL increase
Iron Deficie...
• Parenteral Iron :
Needed = { Body wt in Kg x 2.3 x (15 – pt. Hb)}
+ 500-1000mg for stores
– 500mg at a time/ repeated sm...
Anemia of chronic inflammation
• Iron restricted erythropoesis with
inflammation.
• IL – 1 -> INF ᵞ
• TNF α -> INF β
• Sup...
Anemia of chronic inflammation
• Rx :
– Transfusion : symptomatic/ terminal disease
• Wait up to 7-8 g/dL
• If compromised, maintain at 11 g/dL
• Liberal...
• Recombinant EPO therapy :
– Check for iron stores
– 50-150 U/kg 3 times/week IV
– Up to 300 U/kg, (in chemo induced anem...
Megaloblastic Anemia
Megaloblastic Megaloblastoid
Immature appearing nuclei with large
blast like cells with normal
hemogl...
Megaloblastic Anemia
HYPER SEGMENTED NEUTROPHILS
B12 deficiency Folate Deficiency
S.Cobalamin <100 ng/L S. Folate < 2µg/L
↑ - severe B12 def & Intestinal stagnant
loop syn...
Megaloblastic Anemia
Megaloblastic Anemia
• Rx :
– Treat appropriate vitamin only (always B12 first)
– Transfusion is usually unnecessary & ina...
Hemolytic Anemias
Hereditary Spherocytosis
Family
History
±
Jaundice
Gall stonesSplenomegaly
Mild : Young
adult/ adult
Severe : Infancy
Hereditary Spherocytosis
Hereditary Spherocytosis
↑ MCHC with Normal
blood counts
↑ Osmotic fragility
SDS – gel electrophoresis
of membrane protein...
Hereditary Spherocytosis
• Rx:
– No causal treatment
– Splenectomy
– Mild cases : Defer Splenectomy
– Moderate cases : del...
G6PD Deficiency
• Asymptomatic
• Classical :
– Malaise, weakness, abd & lumbar pain
– In 3 days
– Jaundice & dark urine
• ...
G6PD Deficiency
G6PD Deficiency
• Diagnosis :
– DNA testing
– During hemolytic attack – quantitative test done
for heterozygotes and hemi ...
AIHA
Anemia + Splenomegaly + Jaundice
Warm Ab disease Cold Ab disease
• Ig G1/ G3 mediated
• 37⁰ C
• Extravascular hemolys...
AIHA
AIHA
AIHA
• Medical Emergency, needs transfusion.
• Abs are commonly non specific against ‘e’ Ag of
Rh system.
• So all most al...
PNH
Hemolysis + Pancytopenia + Venous thrombosis
• Classical :
– Early morning bloody urine
• Recurrent severe abdominal p...
PNH
• Reticulocytosis (up to 400,000/µL)
• ↑ MCV, Normo-Macrocytic
• Signs of Hemolysis
• Signs of Iron def (persistent lo...
PNH
• Life long condition
• Filtered RBC (retain WBC)
• S. Iron maintanance
• Folate >3mg/d
• Never long term corticostero...
Aplastic Anemia
• Sudden/ insidious Bleeding (MC 1st symp)
• Easy bruising, gum bleed, nose bleed, heavy menstrual
flow, p...
Aplastic Anemia
• PS : large RBC, paucity of platelets &
granulocytes, ↓↓↓ Retic count
• Immature myeloid forms – Leukaemi...
Aplastic Anemia
Aplastic anemia Hypocellular MDS/Leukemia
• ↓ myeloid/ megakaryocytes
• Normal eyrthroblasts
• ↑ myeloblas...
Aplastic Anemia
• Supportive
• Epoetin/ Darbepoetin + Filgrastim/ Sargramostim
• Transfusions, AMD
Mild
• <40 yrs + HLA ma...
Aplastic Anemia
Immunosuppressive therapy
Initially
• Equine Anti thymocyte globulin (40mg/kg/day x 4)
• Cyclosporin A (6m...
Thalassemia
α- thalassemia β- thalassemia
• (α, α/α,-) Silent carrier (β0/β0 or β +/β +) Thalassemia Major
Transfusion dep...
Thalassemia
• C/f : Onset 4-6 months
• Stunted growth
• Bony deformities
• Hepatosplenomegaly
• Jaundice (gallstones, hepa...
Thalassemia
• Microcytic hypochromic
• 60 – 70 fL MCV
• 22-32 % Hematocrit
• Target cells
• Acanthocytes (irregular spikes...
Thalassemia
Thalassemia
Thalassemia
• Electrophoresis (definitive)
Thalassemia
• Mild – No Rx
• Hb H
– Folate 1mg/day oral
– No oxidative drugs/ iron
– Transfusion
• Splenectomy (if hypersp...
Sickle Cell Anemia
• C/f : Onset 4 – 6 months
– Chronic hemolytic jaundice
– Aplastic crisis with viral illness
– Acute pa...
Sickle Cell Anemia
• Sickled cells (5 – 50%)
• Reticulocytes (10 – 25%)
• Howel jolley bodies (d/t hyposplenism)
• Target ...
Sickle Cell Anemia
Sickle Cell Anemia
Sickle cell anemia
• Allogenic BMT
(Curative in child)
• Omega 3 FA
• Folate 1mg/day
• Transfusions
• Pneumococcal
vaccina...
THANK YOU
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Approach to Anemia - Dr Kasyapa 05-08-16

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Approach to Anemia - Dr Kasyapa 05-08-16

  1. 1. APPROACH TO ANEMIA DR. V. B. KASYAPA. J 1st year GM PG Date : 05/08/16
  2. 2. ERYTHROPOESIS Erythroid/Megakaryocytic Progenitor Erythroid Cells Pronormoblast RBC GATA-1 & FOG-1 EPO 4-5 DIVISIONS
  3. 3. WHO Definition : Anaemia is a condition in which the number of red blood cells (and consequently their oxygen-carrying capacity) is insufficient to meet the body’s physiologic needs.
  4. 4. Clinical Key • Classical symptoms : – Fatigue, Malaise – Dyspnoea on exertion – Loss of stamina – Palpitations – Complaints related to Exertion – Reduced exercise capacity – Pounding sensation in ears – Night sweats • Symptoms suggestive of Inflammation : – Fever – Weight loss
  5. 5. Clinical Key • Symptoms suggestive of Nutritional anemia : – Blood in stools – Loose stools/ Clay colour stools – Constipation – Blood in urine – Pregnancy – Recurrent/Recent foetal loss – History of Neural Tube Defects in offspring – Cleft palate/lip in offspring – Tingling and Numbness of bilateral limbs – Infertility – Prematurity
  6. 6. Clinical Key • Symptoms of Haemolysis : – Yellowish discoloration of eyes and skin – Reversible skin pigmentation • Symptoms suggestive of marrow disorders : – Recurrent upper respiratory tract infections – Episodic jaundice/ abdominal pain/ lumbar pain/ hematuria – Easy bruising/ Gum bleed/ Nose bleed/ Heavy menstrual flow/ Hemiparesis/ Vision loss – Recent viral illness aggravating anemia symptoms – Stunted growth – Priapism
  7. 7. Clinical Key • Symptoms suggestive of genetic disorders : – Early hair greying – Short stature – Dystrophic nails • Past History : – Blood donation history – Phlebotomy history – Drug History – Surgical history – Chemical exposure history – Smoking, Alcoholism
  8. 8. Clinical Key • O/e : – Mental state – Signs of dehydration – Cheilosis (Fissures at corners of mouth) – Koilonychia (Spooning of finger nails) – Pallor ( < 8-9 gm/dL ) • Mucus membranes – Palpebral conjunctiva – Soft palate • Skin – Nail beds – Palmar creases (lighter than surrounding skin when hyperextended)
  9. 9. Clinical Key
  10. 10. Clinical Key – Icterus – Clubbing – Cyanosis – Lymphdenopathy – Pedal edema – Petechiae/ Ecchymosis • Systemic Examination : – Splenomegaly – Hepatomegaly – Forceful Heart beat – Strong peripheral pulses – Systolic Flow murmur – Bony deformities
  11. 11. Clinical Key • Also Look for : – Ascites – Heart failure – Cirrhosis – Endocrinopathies – Pseudoxanthoma elasticum – Neoplasms
  12. 12. Lab Investigations • Complete Blood counts – Hb : Occasion Normal Value (mg/dL) At Birth 17 Childhood 12 Adolescence 13 Adult Man 16 ± 2 Menstruating Adult Female 13 ± 2 Post Menopausal Adult Female 14 ± 2 During Pregnancy 12 ± 2
  13. 13. Lab Investigations – Hematocrit : – Reticulocyte Count – Absolute reticulocyte Count – Recticulocyte Production Index Normal Values (%) Adult Male 47 ± 5 Adult Female 42 ± 5
  14. 14. • RBC Indices • Other Counts – TLC – DLC – Nuclear Segmentation of Neutrophils – Platelet Count Lab Investigations Indices Normal MCV 90 ± 8 fL MCH 30 ± 3 pg MCHC 33 ± 2 % RDW 11.5 – 14.5 %
  15. 15. • Red cell Morphology – Cell size – Mirocytic (<80 fL) – Macrocytic (>100 fL) – Anisocytosis ( Variable size ) – Poikilocytosis ( Variable Shape ) – Polychromasia » ( Slightly larger than normal cells, greyish blue in Wright Giemsa stain » Prematurely released Reticulocytes having residual ribosomal RNA » After EPO stimulation/ architectural damage of BM ) Lab Investigations
  16. 16. • Iron Studies Lab Investigations Study Normal Range Serum Iron 50-150 µg/dL TIBC 300-360 µg/dL Serum Ferritin Males – 100 µg/ L Females – 30 µg/ L Transferrin Saturation 25-50 %
  17. 17. • Marrow Examination – Aspirate : – M/E ratio (1:1) – Cell Morphology – Iron Stain – Biopsy – Cellularity (1:1) – Morphology • Signs of Hemolysis – ↑ Unconjugated bilirubin, urobilinogen, S.LDH – ↓ Haptoglobins – Positive Urine hemsiderin Lab Investigations
  18. 18. Lab Investigation
  19. 19. Lab Investigation
  20. 20. Clinical Classification of Anaemia
  21. 21. Reticulocyte Count • By Supra vital stains – Blue, black punctate spots (precipitated rRNA) • Life – 24 to 36 hrs • Normal range – 1-2% • Daily replacement – 0.8 to 1% • Response depends on – Availability of EPO – Availability of Iron – Healthy Bone marrow
  22. 22. Reticulocyte count
  23. 23. Reticulocyte count • Correction for Anemia – 𝐴𝑏𝑠𝑜𝑙𝑢𝑡𝑒 𝑅𝑒𝑡𝑖𝑐𝑢𝑙𝑜𝑐𝑦𝑡𝑒 𝑐𝑜𝑢𝑛𝑡 = 𝑟𝑒𝑡𝑖𝑐𝑢𝑙𝑜𝑐𝑦𝑡𝑒 𝑐𝑜𝑢𝑛𝑡 𝑥 𝐻𝑏/15 𝑜𝑟 𝐻𝑚𝑡/45 • Correction for prematurely released reticulocytes (only when you see ‘shift’ cells) – 𝑅𝑒𝑡𝑖𝑐𝑢𝑙𝑜𝑐𝑦𝑡𝑒 𝑃𝑟𝑜𝑑𝑢𝑐𝑡𝑖𝑜𝑛 𝐼𝑛𝑑𝑒𝑥 = 𝑅𝑒𝑡𝑖𝑐𝑢𝑙𝑜𝑐𝑦𝑡𝑒 𝑐𝑜𝑢𝑛𝑡 𝑥 𝐻𝑏/15 𝑥 1 / 𝑀𝑎𝑡𝑢𝑟𝑎𝑡𝑖𝑜𝑛 𝑡𝑖𝑚𝑒 𝑐𝑜𝑟𝑟𝑒𝑐𝑡𝑖𝑜𝑛(2)
  24. 24. Clinical Classification of Anaemia
  25. 25. Hypo-proliferative Anemia • Causes : – Mild to Moderate Anemia – Marrow Damage – Inadequate EPO stimulation • Inflammation (IL -1, TNF α, etc..) • Metabolic Disorders ( Hypothyrodism, ..) • Renal Failure ( in DM & Myeloma – marked EPO def seen than actual failure ) • Usually – Normocytic, Normochromic • Marrow is Hypocellular (M/E > 2:1)
  26. 26. Hypoproliferative Anemia Parameter Mild – Mod Iron Def Chr. Inflammation S.Iron ↓ ↓ TIBC ↑↑ ↓/N % Saturation ↓ ↓ S.Ferritin ↓ ↑/N
  27. 27. Maturation Disorder • BM shows erythroid hyperplasia (M/E < 1:1) – But fails to release into peripheral circulation Nuclear maturation defects Cytoplasmic maturation defects Macrocytosis Microcytic, Hypochromic Vit B12/ Folate Deficiency Severe Iron Deficiency Drugs ( Methotrexate/ Alkylating agents/ Alcohol) Globin chain/Heme synth Defects Myelodysplasia
  28. 28. Blood Loss • Normocytic/ Slightly Macrocytic Acute Blood Loss • No Increase in RPI • Signs of Hypovolemia dominate Sub Acute Blood Loss • Modest Reticulocytosis • May show Iron def Chronic Blood Loss • Iron Deficiency picture dominates
  29. 29. Intra Vascular (Iron loss may limit the Marrow response) Acute (Autoimmune hemolysis/ Pathway defects ) Extra Vascular (Efficient recycling of Iron) Chronic (Membrane Defects/ Hemoglobinopathies) Hemolysis
  30. 30. Iron Deficiency Anemia • C/f : – Pregnancy/ Adolescence/ Blood Loss/ Phlebotomy – Advanced tissue iron def » Cheilosis » Koilonychia – In adult male – GI loss, until proven otherwise
  31. 31. Iron Deficiency Anemia • RBC Indices – Microcytic, Hypochromic, Aniso-Poikilocytosis • Iron Studies • Marrow Iron (Normal : 20-40% sideroblasts, with ferritin granules) – Decreases • RBC protoporphyrin Level (Normal : <30µg/dL) – Impaired heme synth (>100 µg/dL).
  32. 32. Iron Deficiency Anemia
  33. 33. DD for Microcytic Anemia
  34. 34. Iron Deficiency Anemia Red cell infusion (Symptomatic/ CV instability/ Elder/ Continued blood loss) Oral Iron (Young/ Asymptomatic) Parenteral Iron (Oral iron intolerance/ Acute need/ Persistent GI loss/ Malabsorption/ EPO therapy Rx
  35. 35. • Oral Iron: – 200mg elemental Iron/day – Absorption will be upto 50mg/d (with good retention capacity) – Supports 2-3x production – Add Ascorbic acid for increased absorption (if cost effective) – 3-4 tabs/day for 6-12 months – GI disturbances (15-20%) – First response : ↑ Retic count in 4-7 days; peak in 7- 10 days Iron Deficiency Anemia
  36. 36. – Iron Tolerance test • 2 Iron tabs on empty stomach • Serial S.Iron for 2 hrs • At least 100 mcg/dL increase Iron Deficiency Anemia Preparation Amount (Elemental Iron) Ferrous sulphate 325 (65) Ferrous Fumarate 325 (107) Polysaccharide Iron 150 (150)
  37. 37. • Parenteral Iron : Needed = { Body wt in Kg x 2.3 x (15 – pt. Hb)} + 500-1000mg for stores – 500mg at a time/ repeated small doses Iron Deficiency Anemia Preparation Amount of Iron per injection Sodium ferric gloconate 125 mg Iron sucrose 200 mg Ferrumoxytol 510 mg Ferric carboxy maltose 750 mg
  38. 38. Anemia of chronic inflammation • Iron restricted erythropoesis with inflammation. • IL – 1 -> INF ᵞ • TNF α -> INF β • Suppresses EPO • Mixed blood picture • Inflammatory component (Normocytic Normochromic) • Iron deficiency (Microcytic Hypochromic) • Acute infection can cause 2-3 g/dL fall in 1-2 days ( d/t death of senile RBC)
  39. 39. Anemia of chronic inflammation
  40. 40. • Rx : – Transfusion : symptomatic/ terminal disease • Wait up to 7-8 g/dL • If compromised, maintain at 11 g/dL • Liberal use in ICU leads to ↑ morbidity & mortality – EPO : Glycoprotein, from peritubular capillary cells in kidney & hepatocytes, regulated by HIF 1 α • Normal – 10-25 U/L; t ½ - 6-9 hrs • Up to 4-5x production in 1-2 weeks Anemia of chronic inflammation
  41. 41. • Recombinant EPO therapy : – Check for iron stores – 50-150 U/kg 3 times/week IV – Up to 300 U/kg, (in chemo induced anemia) – 10-12 g/dL Hb in 4-6 weeks – Stop if acute inf/ iron depletion/ Al toxicity/ hyperpaarthyroidism – Thrombo embolic events, Tumor progression – Long acting : DARBEPOETIN α Anemia of chronic inflammation
  42. 42. Megaloblastic Anemia Megaloblastic Megaloblastoid Immature appearing nuclei with large blast like cells with normal hemoglobinisation. Seen in B12/Folate deficiency. Immature appearing nuclei with defective hemoglobinisation. Seen in Myelodysplasia. CLINICAL FEATURES Anorexia, Wt. loss, Diarrohea/ constipation, Mild fever Tissue effects Hematological effects Mucosal damage Macrocytsis Pregnancy related complications Hyper segmented neutrophil (>5) CVS disorders Leucopenia, thrombocytopenia Malignancies (ALL, Follicular lymphoma, breast & gastric) Hyper cellular marrow CNS abnormalities Signs of hemolysis
  43. 43. Megaloblastic Anemia HYPER SEGMENTED NEUTROPHILS
  44. 44. B12 deficiency Folate Deficiency S.Cobalamin <100 ng/L S. Folate < 2µg/L ↑ - severe B12 def & Intestinal stagnant loop synd S. MMA & Homocystein – Increases S. MMA & Homocystein – Increases B12 absorption tests are obsolete S. Gastrin ↑ S. Pepsinogen I ↓ (for Pernicious Anemia) RBC Folate < 160 µg/L False + in Recent transfusion/ ↑Retic count GI endoscopy & Biopsy (for malabsorption) Diet history Transglutaminase Ab (celiac disease) Duodenal biopsy Megaloblastic Anemia
  45. 45. Megaloblastic Anemia
  46. 46. Megaloblastic Anemia • Rx : – Treat appropriate vitamin only (always B12 first) – Transfusion is usually unnecessary & inadvisable – ↑ platelet count after 1-2 weeks (start aspirin, if >8 lakh/cumm) B12 deficiency Folate deficiency Hydroxy/ cyano cobalamine life long therapy is best Routinue in GIT resections/ longterm PPI therapy Always low threshold for therapy (prevent neuropathy) Oral 5-15 mg/ day For 4 months Replenishment : 6 x 1000 µg IM/ week Maintenance : 1000 µg IM/ 3 monthly Pregnancy Preventive – 400 µg/day Prev NTD history – 4 mg/day Sub-lingual therapy/ Large oral dose (for bleeding disorders)
  47. 47. Hemolytic Anemias
  48. 48. Hereditary Spherocytosis Family History ± Jaundice Gall stonesSplenomegaly Mild : Young adult/ adult Severe : Infancy
  49. 49. Hereditary Spherocytosis
  50. 50. Hereditary Spherocytosis ↑ MCHC with Normal blood counts ↑ Osmotic fragility SDS – gel electrophoresis of membrane proteins Eosin 5’ maleimide (EMA) binding test Acid glycerol Test
  51. 51. Hereditary Spherocytosis • Rx: – No causal treatment – Splenectomy – Mild cases : Defer Splenectomy – Moderate cases : delay until puberty – Sever cases : delay until 4-6 years of age – Anti pneumococcal vaccination before surgery
  52. 52. G6PD Deficiency • Asymptomatic • Classical : – Malaise, weakness, abd & lumbar pain – In 3 days – Jaundice & dark urine • Primaquine mass prophylaxis is a danger • Blood : – Normocytic Normochromic (Mod to Severe) – Heinz bodies ( denatured Hb & hemichromes in supravital staining with methyl violet) – Hemighosts (bizarre, RBC with uneven Hb) – Bite Cell/Blister cell
  53. 53. G6PD Deficiency
  54. 54. G6PD Deficiency • Diagnosis : – DNA testing – During hemolytic attack – quantitative test done for heterozygotes and hemi zygotes • Rx : – No cure – Severe – Transfusions, Splenectomy. – Good prognosis – if No Kidney damage
  55. 55. AIHA Anemia + Splenomegaly + Jaundice Warm Ab disease Cold Ab disease • Ig G1/ G3 mediated • 37⁰ C • Extravascular hemolysis • Phagocytosis • Fragmentation • Cytotoxicity • Acute, Moderate to Severe • Hepatosplenomegaly • Fatigue, SOB • Ig M mediated • 0-4⁰C • Intravascular hemolysis • Membrane attacking complex • Mild to moderate, acute • Acrocyanosis
  56. 56. AIHA
  57. 57. AIHA
  58. 58. AIHA • Medical Emergency, needs transfusion. • Abs are commonly non specific against ‘e’ Ag of Rh system. • So all most all the blood groups will be incompatible • Start incompatible blood transfusion to keep the pt alive (with hold when condition improves) • Prednisolone 1mg/kg/day + • Rituximab 100 mg/week x 4 • Splenectomy, Allogenic BMT.
  59. 59. PNH Hemolysis + Pancytopenia + Venous thrombosis • Classical : – Early morning bloody urine • Recurrent severe abdominal pain (thrombotic event) • Acute hepatomegaly, ascites without liver or cardiac disease • MC COD : Venous thrombosis > Infections (Neutropenia) > Haemorrhage (Thrombocytopenia)
  60. 60. PNH • Reticulocytosis (up to 400,000/µL) • ↑ MCV, Normo-Macrocytic • Signs of Hemolysis • Signs of Iron def (persistent loss) • BM : cellular with massive erythroid hyperplasia • Definitive : ↑susceptibility to complement – Sucrose hemolysis (not reliable) – Acidified serum (Ham) test (Not available) – Flow cytometry (Gold standard) : CD59 -, CD 55- (>5% RBC, >20% granulocytes)
  61. 61. PNH • Life long condition • Filtered RBC (retain WBC) • S. Iron maintanance • Folate >3mg/d • Never long term corticosteroids • ECULIZUMAB IV/ Fort night (↓Compliment mediated hemolysis) – ↑ Extra vascular hemolysis • Allogenic BMT (definitive)
  62. 62. Aplastic Anemia • Sudden/ insidious Bleeding (MC 1st symp) • Easy bruising, gum bleed, nose bleed, heavy menstrual flow, petechia, Hemorrhagic CVA, Retinal hemorrhage • Symptoms of Anemia • History of Drugs (allopurinol, gold, pencillamine)/ Viral illness/ Chemical exposure • Early hair greying – telomeropathy • Peculiar nails & leukoplakia – Dyskeratosis congenita • Café au lait spots & short stature – Fanconi anemia
  63. 63. Aplastic Anemia • PS : large RBC, paucity of platelets & granulocytes, ↓↓↓ Retic count • Immature myeloid forms – Leukaemia/MDS • Nucleated RBC – Marrow fibrosis/ tumor invasion • Abnormal platelets - MDS
  64. 64. Aplastic Anemia Aplastic anemia Hypocellular MDS/Leukemia • ↓ myeloid/ megakaryocytes • Normal eyrthroblasts • ↑ myeloblasts • Abnormal karyotype of erythroblasts Aplastic anemia Hairy cell leukaemia • No splenomegaly • No lymphadenopathy • Splenomegaly • lymphadenopathy
  65. 65. Aplastic Anemia • Supportive • Epoetin/ Darbepoetin + Filgrastim/ Sargramostim • Transfusions, AMD Mild • <40 yrs + HLA matched donor = allogenic BMT • <40 yrs – HLA matched donor/ • >40 yrs =Immunosuppressive therapy Severe (<500/µL neutrophils <20000/µL thrombocytes <1% retic count <20% BM cellularity) • Transfusions • ELTROMBOPAG Refractory
  66. 66. Aplastic Anemia Immunosuppressive therapy Initially • Equine Anti thymocyte globulin (40mg/kg/day x 4) • Cyclosporin A (6mg/kg BD x 6months) • Methyl prednisolone (1-2mg/kg/day x 7day) After 1-3 months • Patient becomes partially transfusion free After 4 months • Complete remission can be seen
  67. 67. Thalassemia α- thalassemia β- thalassemia • (α, α/α,-) Silent carrier (β0/β0 or β +/β +) Thalassemia Major Transfusion dependent • (α,-/α,-) type I α- thalassemia trait • (α, α/-,-) type II α- thalassemia trait Mild Microcytic anemia (β+/β+ with more β chain synth) T. Intermedia Transfusion at aplastic crisis/ stress • (α,-/-,-) Hb H disease Chronic hemolytic anemia of variable severity ± transfusion dependance (β/β0 or β/β+) T. Minor Mild Microcytic hypochromic anemia • (γ, γ/γ, γ) Hb Barts/ Hydrops fetalis Still born
  68. 68. Thalassemia • C/f : Onset 4-6 months • Stunted growth • Bony deformities • Hepatosplenomegaly • Jaundice (gallstones, hepatitis) • Thrombophilia – Over load symptoms • Heart failure • Arrhythmias • Cirrhosis • Endocrinopathies • Pseudoxanthoma elasticum
  69. 69. Thalassemia • Microcytic hypochromic • 60 – 70 fL MCV • 22-32 % Hematocrit • Target cells • Acanthocytes (irregular spikes) • Basophilic stippling (β thalassemias) • Retic count (depends on severity)
  70. 70. Thalassemia
  71. 71. Thalassemia
  72. 72. Thalassemia • Electrophoresis (definitive)
  73. 73. Thalassemia • Mild – No Rx • Hb H – Folate 1mg/day oral – No oxidative drugs/ iron – Transfusion • Splenectomy (if hypersplenism is seen) • Iron chelation – Oral DEFERASIROX 20 – 30mg/kg/day • Allogenic BMT ( Definitive )
  74. 74. Sickle Cell Anemia • C/f : Onset 4 – 6 months – Chronic hemolytic jaundice – Aplastic crisis with viral illness – Acute painful syndrome ( acute vaso occlusion) • Episodes last for hrs to days + low grade fever – Repeated occlusion • Enlarged heart • PAH • Cirrhosis liver • Osteonecrosis (salmonella > staph) • Renal papillary infarction ( gross hematuria) • Retinopathy ( similar to DM) • Auto splenectomy ( initially enlarged ) – Repeated infections – Strokes • Sagittal sinus thrombosis • Priapism
  75. 75. Sickle Cell Anemia • Sickled cells (5 – 50%) • Reticulocytes (10 – 25%) • Howel jolley bodies (d/t hyposplenism) • Target cells • Leukocytosis (12000 -15000/cumm) • Reactive thrombocytosis • Hb Electrophoresis (Definitive)
  76. 76. Sickle Cell Anemia
  77. 77. Sickle Cell Anemia
  78. 78. Sickle cell anemia • Allogenic BMT (Curative in child) • Omega 3 FA • Folate 1mg/day • Transfusions • Pneumococcal vaccination • Iron chelation • Hydroxy Urea 500- 750 mg/OD • Painful crisis : – Hydration, NSAIDS, O2 • Vaso-occlusive/ Intractable pain/ Acute chest synd/ priapism/ stroke – Exchange transfusion Prevention of stroke : 2-16 yr age annual trans-cranial doppler >200 cm/sec velocity ----- start transfusions
  79. 79. THANK YOU

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