Lecture 2 Flashcards by Yara Mohamed

ANEMIA OF CHRONIC DISEASE

Normocytic normochromic

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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

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Pathophysiology of anemia of Chronic inflammation

Inflammation>cytokinesrelease>hepcidin increase>decrease erythropoeitin>decreases usage of iron>decrease life span of RbCs

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HEMOLYTIC ANEMIA

RBCS get broken down before 120 days
It could be due to acquired or congenital disease
It could be extravascular or intravascular

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SEVERE HEMOLYTIC ANEMIA

Rbcs live for 20 days

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Mild hemolytic anemia

40 days

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HEMOLYTIC ANEMIA gets classified according to

LIFE SPAN (MILD,MODERATE,SEVERE)

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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

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Output in hemolysis

Hemoglobin ±uria
+ emia
+haptoglobin decreases
+ hemosideuria (when hemoglobin goes to cell it gives hemosiderin deposit)

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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

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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)

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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

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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)

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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

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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)

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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

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Major thalassemia

Severe anemia
Hemoglobin F or A2 (delta)
Depends on blood transfusion for survival

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Minor thalasemia

Mild anemia
Hemoglobin 7 or 8 or 9
Spleenomegaly
Hemoglobin F or A2
Doesn’t always need blood transfusion

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Intermediate thalassemia

Moderate anemia
Needs blood transfusion in certain situations such as surgery or stress or pregnancy

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2 sheets of alpha affected

Hyochromic anemia

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3 sheets affected

HEMOGLOBIN H
Spleenomegaly and blood transfusion in stressful situations

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4 sheets affected

Parrot hemoglobin=death

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Thalassemia complain

Jaundice even after 6 months from birth

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THALASSEMIA hemoglobin

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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