Lecture 2 Flashcards
ANEMIA OF CHRONIC DISEASE
Normocytic normochromic
Anemia of chronic disease is seen
Collagen disease
Chronic kidney disease
Chronic liver disease
Chron’s
Ulcerative colitis
Systemic lupus
Malignancy(leukemia and lymphoma) by infiltration of bone marrow
Pathophysiology of anemia of Chronic inflammation
Inflammation>cytokinesrelease>hepcidin increase>decrease erythropoeitin>decreases usage of iron>decrease life span of RbCs
HEMOLYTIC ANEMIA
RBCS get broken down before 120 days
It could be due to acquired or congenital disease
It could be extravascular or intravascular
SEVERE HEMOLYTIC ANEMIA
Rbcs live for 20 days
Mild hemolytic anemia
40 days
HEMOLYTIC ANEMIA gets classified according to
LIFE SPAN (MILD,MODERATE,SEVERE)
RBCs when broken down intravascularly release
Hemoglobin that either binds to haptoglobin then release haemoglobinemia
Or binds to hempoxin in kidney and gets filterated there to give hemoglobinemia
Output in hemolysis
Hemoglobin ±uria
+ emia
+haptoglobin decreases
+ hemosideuria (when hemoglobin goes to cell it gives hemosiderin deposit)
HOW TO know HEMOLYTIC ANEMIA ?
Reticulocytosis are high (more than 2%)
INDIRECT/UNCONJUGATED BILIRUBIN IS HIGH
HEMOGLOBINEMIA
HEMOGLOBINURIA
HEMOSIDEROSIS
HDH increases
NORMOCHROMIC NORMOCYTIC (except thalassemia)
HAPTOGLOBIN OR HEMOPEXIN IS LOW
2 types of hemolysis
Intravascular
Or extravascular
(Macrophage takes Rbcs out and accumulates it in liver,spleen, and gall stones so they become enlarged and there will be repeated gall stone)
HEMOLYTIC crisis
Patient with sickle cell anemia travelled and got exposed to hypoxia (abdominal pain,nausea,Vomiting,haematuria,bone pain)
Or patient with favism G6PD deficiency who took drug that got him to crisis
Patient with folic acid and b12 deficiency got into megaloblastic crisis
Or immunocompromised so gets viral infection=APLASTIC crisis
Sickle anemia causes obstruction
To diagnose hemolytic anemia
1) Normochromic normocytic(Except thalasemia)
2) Reticulocytosis more than 2%
3)white blood cells/platelets may increase
4)BONE marrow erythrocyte is high (normoblastic except incase of vitb12 deficiency or megaloblastic anemia)
5)Bilirubin is indirect
6)Haptoglobin decreases and hemopexin decreases
7)LDH increases
8)Blood film(Spherocytosis or thalassemia or sicklecellanemia or unspecific paroxysmal nocturnal hemoglobinuria or autoimmune hemolytic anemia)
Intracorpscular causes of hemolytic anemia
1) inherited (Paroxysmal nocturnal hemoglobinuria)
2)Defect in membrane (spherocytosis/nocturnal hemoglobinuria)
3)defect in globin(thalassemia or sickle cell anemia)
4)defect in enzymes G6PD
Extrinsic
Acquired or
autoimmune or
mechanical :(soilder who stands for 12 hours)
:people who have artifical valves
Or infections like malaria
Or chemicals (organophosphorus/snake or Scorpio bite)
HEREDITARY SPHEROCYTOSIS
*due to loss of spectrin no na/k Atp pump so salt water get retained in rbc so it doesn’t live
*it is familial
*anemia symptoms
*Repeated gallstones
*test osmotic fragility test
*early hemolysis
*treated by splenectomy
Major thalassemia
Severe anemia
Hemoglobin F or A2 (delta)
Depends on blood transfusion for survival
Minor thalasemia
Mild anemia
Hemoglobin 7 or 8 or 9
Spleenomegaly
Hemoglobin F or A2
Doesn’t always need blood transfusion
Intermediate thalassemia
Moderate anemia
Needs blood transfusion in certain situations such as surgery or stress or pregnancy
2 sheets of alpha affected
Hyochromic anemia
3 sheets affected
HEMOGLOBIN H
Spleenomegaly and blood transfusion in stressful situations
4 sheets affected
Parrot hemoglobin=death
Thalassemia complain
Jaundice even after 6 months from birth
THALASSEMIA hemoglobin
F
Blood group of thalassemia
Unknown forever
Bonemarrow of thalassemia
Hyperactive leading to Mongolia features (prominent zygomatic or maxilla)
Organs of thalassemia
Hepatospleenomegaly
Kind of anemia of thalassemia
Microcytic
Thalassemia diagnosis
Hemoglobin electrophoresis hemoglobin F and A2
Or DNA analysis if 1 or 2 sheets only are affected
Differentiation between iron deficiency anemia and thalassemia
In thalassemia mean corpscular volume and iron binding capacity are low
Thalassemia contraindication
IRON
Thalassemia management
Estral+
folic acid+
bone marrow transplantation+
gene therapy+
splenectomy (last resort or if there is huge spleen)
Sickle cell anemia types
SS (most dangerous)
SA
Sickle cell problem
Is a globin problem where one aminoacid gets replaced by another
Patient is normal except in the following cases
Hypoxia
Infection
ACIDOSIS
What happens in sickle cell anemia
Change in shape of Rbc leads to rbcs sticking to each other this blocks veins or leads to venocclosive disease such as abdominal or brain infarction
Spleen of sickle cell anemia
Is small
Symptoms of sickle cell anemia
Severe pain at limbs
Osteomyelitis
Repeated infarctions(stroke or myocardial)
Retina may lead to blindness
Immunocompromised
Ulcer
Repeated gall stones
Avascular necrosis
Since sickle cell patients are immunocompromised
They have to take vaccines such as
Pneumoconiosis influenza virus and meningococci
DIAGNOSIS OF SICKLE CELL PATIENT
Normocytic normochromic anemia
Reticulocytosis
LDH increases
Hemoglobin electrophoresis
Hemoglobin electrophoresis variants
Hemoglobin S (sickle )
Hemoglobin SS (most dangerous)
Hemoglobin SA (least dangerous)
Hemoglobin Sand F (sickelcell anemia and thalassemia)
Treatment of sickle cell anemia
Vaccine
Bone marrow transplantation (when needed)
Gene therapy
Folic acid
Blood transfusion
During crisis give oxygen in case of hypoxia and pain killer and hydroxurea drug (chemotherapy)
Acute chest syndrome
1)Occurs in patients with sickle cell anemia due to venoocclosive diseases
2)They will be in a hypoxic situation,will have fever and leukocytosis
3)infiltration will be seen in X ray
4)poor prognosis
5)symptoms are like respiratory chest syndrome/COVID
GLUCOSE 6 PHOSPHATE DEFICIENCY
The enzyme that produces glutathione to prevent Rbcs from oxidation is deficient
GLUCSE 6 PD is also called
Favism
GLUCOSE 6 PD is
Hereditary and found in Africa
Contraindications for g6pd patients
Legumes
Sulfur containing drugs
Antimalarial drugs(quinolones)
Aspirin
Phenytoin
Lab findings
LDH high
Reticulocytosis high
But everything else is low
Autoimmune hemolytic anemia
A person of 45 years of age
A male or a female with jaundice
Pallor
Patient will have rheumatoid arthritis/Systemic lupus or lymphoma
Key of autoimmune hemolytic anemia
Coombs test IgM IgG
Investigations of autoimmune hemolytic anemia
Reticulocytosis
Combs test
Blood film
Causes of autoimmune hemolytic anemia
Worm time IgG (37)
Idiopathic (lymphoma,lupus,leukemia)
Alpha methyl dopa
Symptoms of hemolytic anemia
Hemolytic anemia and huge spleen
Evan syndrome
Hemolytic anemia+pregnant+thrombocytopenia
Autoimmune disease treatment
Prednisone
Azathioprine
Cyclosporin
Immunosuppressant
Monoclonal antibodies
If all of them fail=splenectomy
Combs test false positive
37degree and above may be due to infectious mononucleosis
Drugs that may cause autoimmune hemolytic anemia
Alpha methyl dopa
Cephalosporin
Penicillin
Antimalarial drugs
Or drugs contraindicated in G6pd
Paroxysmal Nocturnal hemoglobinuria
It’s a stem cell disease
One protein in cell membrane is affected
Paroxysmal nocturnal hemoglobinepuria complain
Wake up with blood in urine
Associated diseases of paroxysmal nocturnal hemoglobinuria
Venocclusive diseases (abdominal pain and Mesenteric infarction and recurrent infection)
Investigation of paroxysmal nocturnal hemoglobinuria
HANS
FLOW CYTOMETRY CD55
Treatment of paroxysmal nocturnal hemoglobinuria
Anticoagulant and blood transfusion