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Bariq. j .Hassan
Wael mohan
Anemia is defined as a low haemoglobin (Hb) concentration,
and may be due either to a low red cell mass or increased
plasma volume (eg in pregnancy).

Normal Hb concentration:
a. 14 to 18 g/dL in men
b. 12 to 16 g/dL in women.
Clinical features

1. A variety of nonspecific
complaints :headache, fatigue, poor
concentration, diarrhea ,nausea ,
dyspnea, vague abdominal
2. Pallor—best noted in the
3. Hypotension and tachycardia
4. Signs of the underlying cause , ‘Conjunctival pallor’, the classic sign of 
jaundice if hemolytic anemia, blood anaemia, is a confusing term as the
conjunctiva is translucent, transmitting the
in stool if GI bleeding. colour of structures under it. The ‘pallor’
refers to the vasculature on the inner surface
of the lid which is lacking Hb.

 Hb
 If Hb reveals anemia,what next ???
 reticulocyte count and MCV.
 Inan anemic patient the first step is to assess
hemodynamic stability. If unstable, transfuse PRBCs
before attempting to find the cause of the anemia.
Hemolytic anemia

1. Anemia that results when RBC lifespan is shortened and marrow

production of RBCs is not capable of meeting demand for new cells
2. Can be caused by defects in RBC membrane, RBC enzyme defects,
hemoglobinopathies, or extracellular effects.
3. History and physical (H/P) pallor, jaundice ; severe cases may
have palpitations, syncope, hepatosplenomegaly , and brownish
discoloration of urine.
4. Labs
 decreased Hgb
 increased reticulocyte count
 increased bilirubin (indirect)
 normal MCV
 Coombs test is helpful for making diagnosis
5. Blood smear schistocytes (RBC
fragments), spherocytes, and/or burr cells.
Iron-deficiency anemia

 Iron deficiency results from blood loss , poor dietary

intake or absorption from the (GI) tract, pregnancy or
menstruation , Colon cancer is a common cause of GI
bleeding in the elderly.
 Labs
 decreased Hgb
 decreased MCV
 decreased or normal reticulocyte count
 decreased ferritin
 decreased iron
 Blood smear microcytic hypochromic RBCs
 Treatment: transfusion if sever anemia,
iron supplementation
(eg ferrous sulfate 200mg/8h PO) , determine cause of iron
Folate-deficiency anemia

1. Anemia resulting from inadequate folate intake, increased

folate need (e.g., poor Nutrition , chemotherapy), or drug-
induced folate metabolism defects (e.g., methotrexate
,trimethoprim, phenytoin)
2. sore tongue , no neurologic symptoms
3. Labs
 decreased Hb
 increased MCV
 decreased reticulocyte count
4. Blood smear macrocytic RBCs, hypersegmented
5. Treatment :Assess for an underlying cause. Treat
with folic acid 5mg/day PO for 4 months + B12 unless
the patient is known to have a normal B12 level.
Vitamin B12 deficiency anemia
 Pernicious anemia (i.e., autoimmune anemia owing to lack of
intrinsic factor) or anemia resulting from inadequate vitamin B 12
intake, ileal resection, bacterial overgrowth in GI tract.
 Symmetric paresthesias, ataxia , possible psychosis
 Labs
 decreased Hgb
 increased MCV
 decreased vitamin B 12
 chilling test useful to diagnose pernicious anemia .
 Blood smear macrocytic RBCs, hypersegmented
 Treatment :Treat the cause if possible. If a low B12 is
due to malabsorption, injections are required.
Replenish stores with hydroxocobalamin (B12) 1mg IM
alternate days, eg for 2wks. Maintenance: 1mg IM
every 3 months for life
Anemia of chronic disease
 Anemia occurring in patients with neoplasia, diabetes mellitus,
autoimmune disorders, or long-standing infections
 H/P history of appropriate disease state, fatigue, weakness,
dyspnea on exertion; tachycardia, pallor
 Labs
• mildly decreased Hgb
• normal or decreased MCV
 Blood smear normocytic RBCs
 Treatment : treat underlying disorder ; supplemental
Aplastic anemia
1. Pancytopenia resulting from bone marrow failure
2. Due to drugs (e.g., chloramphenicol, sulfonamides, phenytoin,
chemotherapeutics) ,toxins, radiation, viral infection, or idiopathic
and congenital causes.
3. H/P persistent infections, poor clotting with possible
uncontrolled bleeding, easy bruising, persistent menstruation;
pallor, petechiae.
4. Labs
• decreased Hb
• decreased (WBCs)
• decreased platelets
• bone marrow biopsy shows hypocellularity and fatty
5. Treatment stop offending agent; transfusions for acute
anemia and thrombocytopenia; immunosuppressive agents
and bone marrow transplant indicated to improve long-term
Sideroblastic anemia
1. Anemia caused by defective heme synthesis resulting in decreased Hgb levels in cells
2. Can be a genetic disorder or caused by alcohol, isoniazid, or lead poisoning (patient history
is useful for differentiating cause)
3. H/P fatigue, weakness, dyspnea on exertion, angina; pallor, tachycardia, tachypnea,
increased pulse pressure, hepatosplenomegaly.
4. Labs
• decreased Hgb
• increased ferritin
• increased iron
• Decreased transferrin
• possible decreased MCV .
5. Blood smear multiple sizes of RBCs with normocytic, microcytic , and
macrocytic cells possible; ringed sideroblasts (RBC precursors) in the bone
6. Treatment
a. Hereditary cases: vitamin B 6
b. Acquired cases: supplemental erythropoietin
c. Both types: iron overload requires deferoxamine ,transfusion may be
required in severe cases
7. Complications 10% patients progress to acute leukemia

 β-chain production is deficient, but the synthesis of α-chains is unaffected

1. Thalassemia major (Cooley anemia; homozygous β-chain thalassemia)occurs
predominantly in Mediterranean populations.
a. Clinical features
• Severe anemia ,Massive hepatosplenomegaly
• Expansion of marrow space—distortion of bones
• Growth retardation and failure to thrive
• If untreated ,death occurs within the first few years of life secondary to
progressive CHF
• Skull x-ray may show “crew-cut” appearance
b. Diagnosis
•electrophoresis—Hb F and Hb A2 are elevated
• Peripheral blood smear—microcytic hypochromic
anemia, target cells may be seen
1.frequent PRBC transfusions are required to sustain
2.Iron-chelators to prevent iron overload, Oral
deferiprone + desferrioxamine sc twice weekly
3.Splenectomy if hypersplenism persists with
increasing transfusion requirements,this is best
avoided until >5yrs old due to risk of infections
2. Thalassemia minor (heterozygous β-chain thalassemia)
a. Clinical features: patients are usually asymptomatic. A mild microcytic,
hypochromic anemia is the only symptom
b. Diagnosis: hemoglobin electrophoresis
c. Treatment: usually not necessary
3. Thalassemia intermedia
a. Usually involves both β-globin genes
b. Severity of anemia is intermediate
c. Patients usually are not transfusion dependent.
There is a decrease in α-chains, which are a component of all types of hemoglobins.
1. Silent carriers—mutation/deletion of only one α-locus
 Asymptomatic, Normal hemoglobin and hematocrit level, No treatment necessary
2. α-Thalassemia trait (or minor)—mutation/deletion of two α-loci
 Characterized by mild microcytic hypochromic anemia,No treatment necessary
3. Hb H disease—mutation/deletion of three α-loci
a. Hemolytic anemia, splenomegaly
b. Significant microcytic, hypochromic anemia
c. Hemoglobin electrophoresis shows Hb H
d. Treatment is often the same as for patients with β-thalassemia major.
4. Mutation/deletion of all four α-loci—this is either fatal at birth (hydrops fetalis) or
shortly after birth.
Sickle cell disease

 Autosomal recessive defect in globin chain of Hgb, leading to production of

abnormal Hgb S .
 Acidosis, hypoxia, and dehydration cause Hgb S molecules to polymerize and
distort RBCs into a sickle shape that is more susceptible to hemolysis and
vascular clumping than normal cells.
 H/P sickle cell crisis characterized by deep bone pain , chest pain, new
stroke onset, painful swelling of hands and feet, dyspnea, priapism (i.e.,
painful, prolonged erection); growth retardation, splenomegaly, jaundice,
fever, tachypnea, leg ulcers seen on examination
 Labs
a. increased reticulocyte count
b. increased polymorphonuclear (PMN) cells
c. increased bilirubin (indirect)
 Hemoglobin electrophoresis detects Hgb S without normal Hgb A;
Hgb F may be increased.
 Radiology “fish-mouth” vertebrae; lung infiltrates in acute chest
 Blood smear target cells, nucleated RBCs.
 Treatment
a. Hydration, supplemental O 2 , and analgesics (frequently narcotics
required) during sickle cell crises
b. Hydroxyurea (increases Hgb F production)
c. Pneumococcal vaccine ,prophylactic penicillin should be given until
5 years.
d. Chronic transfusions.
e.stem cell transplantation and gene therapy show future promise as
potential cures.
Congenital coagulopathy

• Von Willebrand disease is the most common congenital bleeding

disorder ,characterized by deficient or defective (vWF), which is
essential for platelet adhesion. Treatment of von Willebrand disease is
with desmopressin (DDAVP) ,(rVWF).
• Hemophilia A (classic hemophilia) is caused by factor VIII deficiency.
Severe bleeding is common.
• Hemophilia B (Christmas disease) is due to a factor IX
deficiency. Treat hemophilia A with factor VIII concentrate. Treat
hemophilia B with factor IX concentrate. The dosage is based on the
site of the bleeding.
Acute anemia due to blood loss

• Traumatic injury, massive upper or lower gastrointestinal

(GI) hemorrhage, ruptured ectopic pregnancy, ruptured
 Monitor with pulse oximetry, cardiac monitor, and a
sphygmomanometer. Provide supplemental oxygen via face mask.
Establish 2 large-bore intravenous (IV) lines, and rapidly infuse 1-2 L
of crystalloid while monitoring the patient carefully for signs and
symptoms of iatrogenic congestive heart failure.
 Consider type O or type-specific blood transfusion for patients who
remain hypotensive after 2 L of crystalloid infusion; this applies to
young healthy patients with a hematocrit below 20% and elderly
patients with a hematocrit below 30%.
 Vasopressors are relatively contraindicated in the treatment of
hypovolemic shock.
Thank you