2. HAEMOPOIETIC SYSTEMBLOODPlasma Coagulation factorsCells White
blood cells/Leukocytes (WBCs) Red blood cells/Erythrocytes (RBCs)
Platelets/Thrombocytes 3. HAEMOPOIETIC SYSTEMHaemopoiesisyolk
sacLiverSpleenBone marrow long bones (children) Axial skeleton
(adults) 4. HAEMOPOIETIC SYSTEMDISEASES OF HAEMOPOIETIC
SYSTEMCytopenia production destruction /
poolingCytosisreactivemalignant 5. HAEMOPOIETIC SYSTEMRBCs
AnaemiasWBCs Leukaemias myeloproliferative disorders Lymphomas
plasma cell disordersPlatelets Thrombocytopenia function
defectsCoagulation disorders factor deficiency 6. HAEMOPOIETIC
SYSTEMANAEMIA:Reduction below the normal limits of thetotal
circulating red cell mass.Reduction in Hb, HCT/PCV below thenormal
limit for that age, sex andenvironment 7. HAEMOPOIETIC
SYSTEMERYTHROPOIESIS:Formation of RBCsErythropoietinMinerals,
vitamins etc:iron, Mn, Covit B12, folic acid, B6, vit C, E, B1amino
acidshormones 8. HAEMOPOIETIC SYSTEMCLASSIFICATION OF
ANAEMIAS:Morphological Size Shape ColourPathogenetic blood loss
Reduced production increased destruction 9. HAEMOPOIETIC
SYSTEMMorphological classification:1. Normocytic Normochromicblood
lossaplastic anaemiaACDhemolytic anameia 10. HAEMOPOIETIC SYSTEM2.
Microcytic HypochromicIron deficiency
anaemiaThalassaemiasSidroblastic anaemiaACD 11. HAEMOPOIETIC
SYSTEM3. Macrocytic Megaloblastic anaemia hemolytic anaemia 12.
HAEMOPOIETIC SYSTEMPathogenetic classification:1. Blood loss Acute
Chronic2. Increased destruction (hemolytic) Hereditary membrane
defectsSpherocytosisElliptocytosis 13. HAEMOPOIETIC SYSTEM
Metabolic defectsG6PD deficiencyPK deficiency Hb defectsquantitaive
defectsThalassaemiasqualitative defectssickle cell anaemiaHbC, D,
Eunstable Hb 14. HAEMOPOIETIC SYSTEMAcquired Immune hemolytic
anaemias Non immuneMAHAs (mechanical)PNHmalaria & other
infectionsmechanical trauma Hypersplenism DIC Drugs 15.
HAEMOPOIETIC SYSTEM3. Impaired production: Nutritional
deficienciesmegaloblastic anaemiairon deficiency anaemia Aplastic
anaemia Pure red cell aplasia sideroblastic anaemia anaemia of
chronic disease 16. HAEMOPOIETIC SYSTEMParameter Males
FemalesHb15+/-2 gm/dl13+/-1.5RBC count 5+/-0.5x10 /l 124.5+/-0.5PCV
0.45+/- 0.05l/l 0.4+/- 0.5 l/lMCV 92 +/- 9 flMCH 29.5+/-2.5
pgMCHC33 +/- 1.5 g/dlCell diameter 6.7 7.7
umReticulocyte0.5-2.5%count 50 100x10 /l 12 17. HAEMOPOIETIC
SYSTEMHEMOLYTIC ANAEMIAS:Normal red cell life span = 120
daysSplenic reticuloendothelial cellsDegradation of Hb bilirubin
18. HAEMOPOIETIC SYSTEMHemolysis shortened red cell life
spanStimulated erythropoiesis intra &extramedullary Hb
breakdown bilirubin jaundice gall stones 19. HAEMOPOIETIC
SYSTEMExtravascularIntravascularPhysiologicPathologicRE cells
spleenIn blood vesselsFc receptor mediated Complement mediatedLess
deformable RBCMechanical injurySplenomegaly ++Splenomegaly
+/-Indirect Hbaemia,
Hburia,hyperbilirubinaemiahemosidrinuriaJanudice ++Jandice +Normal
plasma Haptoglobin 20. HAEMOPOIETIC SYSTEMAnaemia EPO erythroid
hyperplasia Marrow expansion reticulocytosis Leukoerythroblastic
blood picture Gall stones haemosiderosis 21. Never look down on
anybody unless youre helping him up 22. HAEMOPOIETIC SYSTEM
HEREDITARY SPHEROCYTOSIS: 23. HAEMOPOIETIC SYSTEMAutosomal
dominantIntrinsic membrane defectDeformability of RBC due to
cytoskeletalProteinsSpectrinActinAnkyrinBand 4.2 & 3 24.
HAEMOPOIETIC SYSTEMReduced membrane stabilityReduced deformability
spheroidalTrapped in spleen lactic acid ++ Intracellular Na+
osmotic injuryPhagocytosis by RE cellsSplenomegaly 25. HAEMOPOIETIC
SYSTEMVariable severityChronic hemolytic anaemiaAplastic
crisisHemolytic crisis 26. HAEMOPOIETIC
SYSTEMDIAGNOSIS:HistoryExaminationBlood filmOsmotic Fragility test
27. HAEMOPOIETIC SYSTEMGLUCOSE 6 PO4 DEHYDROGENASEDEFICIENCY 28.
HAEMOPOIETIC SYSTEMX linkedOxidant damage to RBCs Heinz bodies Bite
cellsOxidant stress: Drugs (antimalarials, sulfonamides) Foods
(Fava beans) Infections 29. HAEMOPOIETIC SYSTEMVariants G6PD-A
(Mediterranian) G6PD-B (commonest) G6PD A- (African, mild)Neonatal
jaundiceAcute hemolysisChronic low grade hemolysisIntravascular
hemolysisExtravascular hemolysis 30. HAEMOPOIETIC SYSTEM5 years
boyProgressive pallorPainful swelling of fingers & toesJaundice
twiceO/E pallor ++Jaundice +Spleen +Leg ulcersShort right middle
finger 31. HAEMOPOIETIC SYSTEMHb = 5.5 gm/dlMCV = 74 flMCH = 26
l/lTLC = 18.6Plt = 110Retics = 12 %Leucoerythroblastic blood
picture 32. HAEMOPOIETIC SYSTEMHAEMOGLOBIN ELECTROPHORESIS: Band of
HbS 33. HAEMOPOIETIC SYSTEM SICKLING TEST 34. HAEMOPOIETIC
SYSTEMSICKLE CELL DISEASE:Autosomal recessiveStructural variant of
Hb6th position globin chainValine Glutamic acid HbS 35.
HAEMOPOIETIC SYSTEM Deoxy Hb Crystallization/polymerization HbS
Tactoid formation (reversible) Repeated sickling de-sickling
Irreversibly sickled Vascular occlusion 36. HAEMOPOIETIC SYSTEM
Membrane damage Ca K+ Intracellular dehydration Sticky RBCsVascular
occlusion 37. HAEMOPOIETIC SYSTEMHomozygous SCDHeterozygous SC
traitHbF inhibits polymerization of HbSIntracellular dehydration
MCHC Sickling ++ pH deoxy Hb sickling 38. HAEMOPOIETIC
SYSTEMCLINICAL PRESENTATION:Chronic hemolysis
(extravascular)AnaemiaJaundiceInfections by encapsulated organisms
osteomyelitis (salmonella)H-Influenza, PneumococciLeg ulcers 39.
HAEMOPOIETIC SYSTEMVaso occlusive crisis painful crisisbones hand
foot syndromelungs acute chest syndromebrain seizures / strokeliver
hepatic sequestration painspleen sequestration syndromepenis 40.
HAEMOPOIETIC SYSTEMMarrow expansion hair on ends(x-ray
skull)Prominent cheek bonesExtramedullary haemopoiesisGall
stonesAplastic crisis (parvo virus)Autosplenetomy 41. HAEMOPOIETIC
SYSTEMDIAGNOSIS:HistoryExaminationPeripheral
smearISCleucoerythroblastic blood picturereticulocytosis 42.
HAEMOPOIETIC SYSTEMHb electrophoresis HbS bandSickling testPCR 43.
HAEMOPOIETIC SYSTEMMANAGEMENT:Blood transfusion (RCC)PreventionBone
marrow transplant 44. A man who is "of sound mind" is one who keeps
the inner madman under lock and key 45. HAEMOPOIETIC
SYSTEMHAEMOGLOBIN:Heme + 4 Globin chainsHbA 2a/2HbA2 2a/2HbF 2a/2
and embryonic chains 46. HAEMOPOIETIC SYSTEMSynthesis of globin
chains: Haemopoietic GF Gene activation Transcription Translation
Post translation stability 47. HAEMOPOIETIC SYSTEM1.5 year
girlFailure to thrivepallor and abdominal distensionCousin died at
3 years with similar problemsOne transfusion 2 months backO/E
pallor +++Spleen 4 cmLiver edge 48. HAEMOPOIETIC SYSTEMTLC :24.6Hb
: 6.2Plt :130MCV :72MCH :21NRBC 43/100 WBCLeft shift in neutrophils
49. HAEMOPOIETIC SYSTEMHbF :97%DIAGNOSIS:Clinical, blood and
electrophoresis findings are consistent with - THALASSAEMIA MAJOR()
50. HAEMOPOIETIC SYSTEMTHALASSAEMIAS:Reduced or no synthesis of one
or moreglobin chains of Hb.Autosomal recessive chain deficiency
thalassaemia chain deficiency thalassaemia 51. HAEMOPOIETIC
SYSTEMCLASSIFICATION:ClinicalGeneticThalassaemia major homozygous +
(+/ +) homozygous o (o / o) / Hb Lepore / HbE 52. HAEMOPOIETIC
SYSTEMThalassaemia Intermedia: +/+ with thalassaemiaHbH disease /
compound heterozygotesHbE/ 53. HAEMOPOIETIC SYSTEMThalassaemia
minor:Silent carriers + thalassaemia trait rare thalassaemia
traitMild anaemia o thalassaemia trait+/+ thalassaemia o trait +
trait trait 54. HAEMOPOIETIC SYSTEMBETA THALASSAEMIA:Mutations in
globin geneTranscription promoter region mutations chain terminator
mutationsProcessing of mRNA splicing mutations splice site in exon
IVS CAP site 55. HAEMOPOIETIC SYSTEM Translation nonsense
frameshift initiation site Post translational stability Exon 3 56.
HAEMOPOIETIC SYSTEMDIAGNOSIS:HistoryExaminationBlood filmHb
electrophoresisPCRFamily studies 57. HAEMOPOIETIC
SYSTEMTREATMENT:Regular blood transfusionsIron chelation injectable
oralSupportive treatmentBone marrow transplant 58. HAEMOPOIETIC
SYSTEMPREVENTION***genetic counselingantenatal
diagnosisCOMPLICATIONS:growth retardationiron overload endocrine
abnormalities CCF hepatic failuretransfusion mediated infections
(HBV,HCV) 59. "Sometimes the best helping hand you canget is a
good, firm push 60. HAEMOPOIETIC SYSTEMACQUIRED HEMOLYTIC
ANAEMIAS:Immune hemolytic anaemia:Autoimmune HAwarm antibody type
idiopathic autoimmune diseases LPD infections cancers drugs 61.
HAEMOPOIETIC SYSTEM cold antibody type cold agglutinin syndrome
CHAD (idiopathic) infections LPD PCH 62. HAEMOPOIETIC
SYSTEMAlloimmune HA transfusion reactions HDN allograft associated
drug inducedmacrophage mediatedcomplement mediated 63. HAEMOPOIETIC
SYSTEMWarm antibody HA Cold antibody HACommonest (45-70%) 15-30%50%
idiopathic Secondary >IgG, IgA IgMExtravascularIntravascular
++hemolysisExtravascular 100 flpancytopeniahypersegmented
neutrophils 83. HAEMOPOIETIC SYSTEMBone marrow
findings:HypercellularDyserythropoiesisIneffective
erythropoiesisMegaloblastsGiant myeloid precursorsHyperlobated
megakaryocytesAbnormal mitotic figures 84. MEGALOBLAST:Large
abnormal erythroid precursorsnuclear cytoplasmic asynchrony 85.
HAEMOPOIETIC SYSTEMCOBALAMINE (VITAMIN B12)Only source animal
foodBody stores = 2-3 mgSufficient for 3-4 yearsAbsorption (ileum,
IF)Transport (transcobalamines) 86. HAEMOPOIETIC SYSTEMCauses of
Cobalamine deficiency:1. Vegans2. Malabsorption Pernicious anaemia
gastric causesIF defgastrectomy 87. HAEMOPOIETIC SYSTEM intestinal
causesstagnant loop syndromesileal resectioncrohns diseasetropical
spruefish tape wormsTC deficiency 88. HAEMOPOIETIC SYSTEMPERNICIOUS
ANAEMIASpecific type of MBASevere lack of Intrinsic factor due
togastric atrophyAutoimmune disorderAnti IF antibodiesAnti Parietal
cell antibodies 89. HAEMOPOIETIC
SYSTEMDiagnosis:HistoryExaminationBlood CPBone marrow
examinationSerum B12 levelsSchillings testIF & PC
antibodiesElevated homocystine & CH3-malonic acidTherapeutic
response 90. HAEMOPOIETIC SYSTEMFOLIC ACID (Pteroylglutamic
acid)Liver, yeast, spinach, greens, nutsTotal store = 10 mgFor 4-6
monthsAbsorbed from upper small intestinePolyglutamates
monoglutamatesTransported by albumin 91. HAEMOPOIETIC SYSTEMCauses
of deficiency:1. Dietary: old age, poverty, infancy, alcoholism,
psychiatrics2. Malabsorption tropical sprue celiac disease
intestinal resection crohns disease 92. HAEMOPOIETIC SYSTEM3.
Excess demand: physiologicalpregnancy, prematurity, lactation
pathologicalhemolytic anaemias, malignanciesinflammatory
conditionshomocystinuriahemodialysis patients 93. HAEMOPOIETIC
SYSTEM4. Anti-folate drugs: anticonvulsants antiTB tetracyclins5.
Liver disease, alcoholism 94. HAEMOPOIETIC SYSTEMTreatment:1. Of
cause2. Cobalamine injections3. Folic acid tablets 95. HAEMOPOIETIC
SYSTEMAPLASTIC ANAEMIA:Presence of pancytopenia in theperipheral
blood & a hypocellularmarrow in which normal haemopoieticmarrow
is replaced by fat cells. 96. HAEMOPOIETIC SYSTEMHaemopoietic stem
cell defectDefect in microenvironmentCytotoxic T cell mediated
suppression ofStem cellsIFN gamma, TNFAbsence of abnormal cells or
fibrosis inBone marrow 97. HAEMOPOIETIC SYSTEMCauses:Idiopathic
primary stem cell defect immune mediatedChemical agents dose
relatedalkylating agents & antimetabolitesbenzene,
arsenicchloramphenicol 98. HAEMOPOIETIC SYSTEMidiosyncratic
chloramphenicol phenylbutazone arsenic, streptomycin insecticides
& pesticidesPhysical agentsradiation 99. HAEMOPOIETIC
SYSTEMInfections hepatitis CMV, EBV, herpes VInherited Fanconis
anaemia 100. HAEMOPOIETIC SYSTEMClinical presentation:Anaemia
Thrombocytopenia Neutropenia 101. HAEMOPOIETIC
SYSTEMDiagnosis:HistoryExaminationBlood CPBone marrow aspiration
& trephine 102. The purpose of life is to live a life of
purpose. 103. HAEMOPOIETIC SYSTEM 104. HAEMOPOIETIC SYSTEM 105.
HAEMOPOIETIC SYSTEM 106. HAEMOPOIETIC SYSTEM 107. HAEMOPOIETIC
SYSTEM 108. "You make the world a better place by making yourself a
betterperson." 109. HAEMOPOIETIC SYSTEM
