Approch to patient hemolytic anaemia.pptx

DrsoadAdel 48 views 34 slides Sep 21, 2024
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About This Presentation

type of anemia result from an increase in the rate of red cell destruction. which leads to reduction in the mean lifespan of the red cell
In a healthy person, a red blood cell survives 90 to 120 days in the circulation,
 In conditions where the rate of RBC breakdown is increased, the body initia...


Slide Content

Approch to hemolytic anaemia

Deffention of anemia

Inability of red blood cell to carry oxygen from lung to be utilized in other body tissues due to decrease red blood cell mass Normal values of hb :- Hb …….male 13.5-17.5 Hb…..female 11.5-15.5

Classification of anemia According to morphology and size of rbc

According to etiology

Impaired production : 1- nutrient deficiency : Iron deficiency anemia Megaloblastic anemia * folate and vit b 12 deficiency 2- chronic disease : Renal disease . Liver disease . 3- sideroblastic anemia 4- aplastic anemia

2- Excessive distruction : (hemolytic anemia ) Intrensic factors ( intracorascular ) Cell membrane : *hereditary spirocytosis * hereditary elliptocytosis Hemoglobin Thalassemia Sickle cell Enzymes G6PD anemia Pyruvate kinase

Extrensic factors ( extracorpascular ) Immune : auto immune allo immune drug induced Mechanical : macro angiopathic Cardiac Direct effect of physical or chemical or infectious Hyper spleenism

Hemolytic anamia

Deffenetion of hemolytic anemia  type of anemia result from an increase in the rate of red cell  destruction. which leads to reduction in the mean lifespan of the red cell

 In a healthy person, a red blood cell survives 90 to 120  days in the circulation,  In conditions where the rate of RBC breakdown is increased, the body initially compensates by producing more RBCs; however, breakdown of RBCs can exceed the rate that the body can make RBCs, and so anemia can develop.  The normal adult marrow, after full expansion, is able to produce red cells at   6-8 times the normal rate and this leads to  reticulocytosis . Therefore  Hemolytic Anemias may not be seen until the red cell lifespan is less than 30 days.

Clinical picture

generalized fatigue Jaundice Fever Spleenomegally Hepatomegly Change in urine colour Gall stone Leg ulcer Bony deformity

classification

Mechanism of hemolysis

How to diagnose????

ASH GUIDLINESS

Diagnostic tests Indication for tests Primary evaluation     Hemolytic screen      FBC, blood smear, LDH, haptoglobin, bilirubin, DAT, reticulocyte count with or without urine for hemosiderin or urine dipstick for microscopy     Detection of underlying disorders (investigation of AIHA)      Serum Igs and electrophoresis     HIV, HBV, HCV      Anti-dsDNA, ANA      CT chest, abdomen, and pelvis    Additional investigation in selected patients with AIHA     Bone marrow examination  CAD, age ≥ 60 years, features in history, examination, FBC or smear suggesting possible marrow infiltration   U&E, LFT, clotting, BP, urine dipstick  If pregnant or thrombocytopenic, to exclude DIC or pregnancy-associated TMA   Infection screening  Dependent on symptoms, travel history, and age   Parvovirus, hematinics  If reticulocytopenia   Thermal range of cold antibody  If clinical significance of cold autoagglutinin unclear 

Additional serological investigation in selected patients with AIHA      DAggT   If DAT positive for C3d ± IgG   Cold antibody titer  If DAggT positive   Monospecific DAT for IgM, G, A, C3d  If DAT-negative AIHA suspected   Red cell eluate  If (monospecific) DAT-negative AIHA suspected   Donath Landsteiner  If DAT is positive for C3d ± IgG and  i ) DAggT -negative or insignificant CAs and 

ANA, antinuclear antibody; BP, blood pressure; CAD, cold agglutinin disease; CAs, cold agglutinins; CT, computerized tomography; DAggT , direct agglutination test; DIC, disseminated intravascular coagulation; dsDNA, double-stranded DNA; FBC, full blood count; HBV, hepatitis B virus; HCV, hepatitis C virus; LFT, liver function tests; TMA, thrombotic microangiopathy; U&E, urea and electrolytes.

Caused by antibody produce by body against himself Charactrized by positive dat ( coombs ) which is divided into warm and cold agglutinin test 15-25% of AIHA was – ve DAT

Classification of immune hemolytic anamia

Warm type Cold type Autoimmune Idiopasic Secondary ( sle,cll,lymphoma ) Drugs(methyl dopa) Idiopasic Secondary Infections ( mycoplasmic pnemina ,infectious mononucleosis) Lymphoma Paroxysmal cold hemoglobinuria Alloimmune Induced by RBCS antigen Hemolytic transfusion reaction Post stem cell graft Drug induce AB react strongly with RBCS at 37*c AB react strongly with RBCS 4*c IgG +/-c3 IgM ttt …..steroid , rituximab.spleenectomy ttt …… rituximab,steroid and splenectomy has no role

Treatment

i.supportive ttt :- 1-good hydration of pt 2- analgesic to control body ache 3- antipyretic in case of high fever 4- pan culture in case of infection 5- antibiotic coverage 6-prophylactic anticoagulant if not contraindicated 7-Blood transfusion if needed
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