Transcript ANEMIAS Dr. Aamer Aleem Consultant Hematologist Associate Prof. of Medicine

ANEMIAS
Dr. Aamer Aleem
Consultant Hematologist
Associate Prof. of Medicine
KKUH & College of Medicine
Riyadh
Anemia
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Anemia is present when a patient has a
reduced amount of hemoglobin per
unit volume of blood when compared
with the correct reference population
for that patient.
Males
 Females
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Hb-13-18
Hb 12-17
ANEMIA
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Single cell line(RBC) problem
Multiple cell line problem
(RBC,WBC,Platelet)
-Bone marrow suppression
-immunologic disorders
-peripheral destruction/sequestration
Anemia
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Anemia is generally defined as a hematocrit
<40% (hemoglobin <13.0 g/dL) in men or
<37% (hemoglobin <12.0 g/dL) in women.
(WHO definition)
Red blood cell (RBC) indices, which include the
mean corpuscular volume (MCV), the mean
corpuscular hemoglobin (MCH), the mean
corpuscular hemoglobin content (MCHC), and
the red-cell distribution width (RDW) index, are
further used to define types of anemia.
Anemia
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Despite having a set of peculiar symptoms
and signs, anemia is not a disease per se,
but a syndrome, as it may arise from an
extensive list of causes.
It is the chronic syndrome of highest
prevalence in clinical medicine.
Anemia
Etiology
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Based on Hb, red cell indices, retic count
and red cell morphology
(1) Inadequate response
A. Hypochromic microcyctic
B. Normochromic Normocytic
C. Macrocytic
(2)Adequate response
R/O blood loss---Includes Hemolytic
disorders
Anemia-symptoms
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What are the symptoms of Anemia ?
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General malaise, weakness, fatigue, breathlessness
on exertion, palpitations, angina.
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Desire to eat sand and clay.
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Menorrhagia common in women.
Symptoms of Anemia
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The central nervous system, the heart and the muscle
mass are the most affected organs, since they are the
ones that most need oxygen for their functions.
The symptoms increase with physical activity, as this
consumes oxygen.
With hemoglobin between 9 and 11 g/dL there's
irritability, headache and psychic asthenia; in the elder
fatigability is observed, and angina may occur.
With hemoglobin between 6 and 9 g/dL there's
tachycardia, dyspnea and fatigue upon the slightest
effort.
With hemoglobin below 6 g/dL symptoms are present
even in sedentary activities, and when below 3.5 g/dL
heart failure is impending and any activity is unfeasible.
COMPLETE BLOOD COUNT
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Hb Conc
(g/dl)
Hematocrit (PCV)
MCV
(fl)
MCH
(pg)
RDW(measure of red cell size variability)
RBC Count
(…x109/L )
WbC Count
(…x109/L )
Platelet Count
(…x109/L)
(Reticulocyte Count) ( % )
Red Cell Indices
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MCV defines the size of the red blood cells and is
expressed as femtoliters (10−15; fl) or as cubic microns
(μm3). The normal values for MCV are 87 ± 7 fl.
MCH quantifies the amount of hemoglobin per red
blood cell. The normal values for MCH are 29 ± 2
picograms (pg) per cell.
MCHC indicates the amount of hemoglobin per unit
volume. In contrast to MCH, MCHC correlates the
hemoglobin content with the volume of the cell. It is
expressed as g/dl of red blood cells or as a percentage
value. The normal values for MCHC are 34 ± 2 g/dl.
Red Cell Indices
How to calculate red cell indices
Glossary of Useful Blood Count
Descriptors
Anisocytosis: red cells of unequal size. Reflected in
increased RDW (Red cell Distribution Width.)
Dimorphic Blood Film: two populations of red cells - one
microcytic and the other normocytic. Seen in treated or
transfused iron deficiency, and sideroblastic anemia
Howell-Jolly bodies: round nuclear remnants within the
red cells. Indicate splenectomy or
hyposplenismMacrocytosis: large red cells Erythroblast:
any nucleated red cell precursor Hypersegmented
neutrophils: a neutrophil with six or more lobes. Usually
(but not inevitably) means vitamin B12 or folate
deficiency
Glossary of Useful Blood Count
Descriptors
Hypochromia: pale red cells. Always accompanied by
microcytosis Leukoerythroblastic: the presence of
erythroblasts and myelocytes (which are precursors of
mature cells) in the blood. Often indicates marrow
infiltration eg by secondary cancer or fibrosis
Microangiopathy: indicates mechanical damage to red
cells with red cell fragments on the blood
filmMicrocytosis: small red cells Poikilocytosis: a
traditional term for red cells of unequal shape
Polychromasia: grey coloured red cells on film, indicating
presence of increased reticulocytes
Reticulocyte: an erythrocyte newly released from the bone
marrow
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Glossary of Useful Blood Count
Descriptors
Rouleaux: red cells in stacks, as coins. Indicates high
ESR, eg infection, myeloma, cancer, collagen disease,
TB etc.
Schistocyte: a red cell which has undergone mechanical
damage - synonymous with red cell fragment
Spherocyte: a spherical red cell due to disproportionate
membrane loss. Either inherited, or acquired from
(usually) immune causes
Sickle cell: a crescent-shaped red cell characteristic of
Sickle Cell Anemia
Target cell: red cell with central area of Hb giving the
appearance of a target. Seen in many conditions,
including IDA, hemoglobinopathy and liver disease
Rouleaux
Rouleaux (singular is rouleau) are
stacks of red blood cells. They occur
when the plasma protein
concentration is high, and because of
them the ESR (erythrocyte
sedimentation rate) is also increased.
Conditions which do this include
infections, inflammatory and
connective tissue disorders, and
cancers. In this case, the diagnosis is
probably multiple myeloma, as a
plasma cell can be seen on the film.
Erythroblasts & other BM cells
Erythroblasts are the
precursor cells of erythrocytes.
They contain nuclei, and in
adults are only found in the
bone marrow under normal
circumstances. Their presence
in the blood may indicate
either marrow 'stress', as in
hypoxia, severe sepsis, or
hemolysis, for example, or
some fundamental bone
marrow pathology such as
replacement by secondary
cancer.
Anisocytosis
Anisocytosis means that the red
cells are of unequal size. It is a
feature of many anemias, and
other blood conditions, and does
not have much diagnostic value.
The 'red cell distribution width'
(RDW) is a quantitative measure
of the degree of anisocytosis.
The RDW is useful in the
differential diagnosis of
microcytic anemia. Most cases
of iron deficiency have a raised
RDW, and most cases of
thalassemia trait have a normal
RDW.
Dimorphic Red Blood Cells
There are two
populations of red cells
present. One is
normocytic, and the
other is microcytic. This
occurs either because an
iron-deficient patient
has been transfused or
treated with iron, or in
the Sideroblastic
Anemias.
Anemia-diagnosis
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How can we diagnose Anemia ?
Blood tests to detect the various components of
blood is carried out for proper diagnosis.
Red blood cell count may be normal or decreased.
Peripheral blood smear shows pale small cells.
White blood cell count normal or decreased
Classification of anemia
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Anemia can be either acute or chronic.
In acute anemia (sudden loss of blood), the lack of blood
volume in the circulatory system is more important than
the deficiency of hemoglobin. A loss up to 10% of blood
volume, as that taking place upon blood donation, is well
tolerated. Losses between 10 and 20% cause postural
hypotension, dizziness and faint. In losses above 20%,
there's tachycardia, cold extremities, extreme paleness
and hypotension, followed by shock; should the loss
surmount 30%, without immediate replacement of
intravenous fluids, the shock rapidly becomes
irreversible and fatal.
In chronic anemia, there's no decrease in blood volume,
which is compensated by an increase in plasma volume.
Classification of anemia
Based on MCV
 Microcytic anemia
 Normocytic anemia
 Macrocytic anemai
Classification of anemia
Based on underlying process
 Blood loss or def. of nutrients
 Hemolysis
 Failure of production
A diagnostic scheme for anemias
Determine the MCV
 The most useful initial approach to anemia
is based on red cell size. The Mean
Corpuscular Volume (MCV) represents a
direct measurement of red cell size. When
this is reduced, the anemia is referred to as
microcytic. It is macrocytic when the MCV
is increased, and normocytic when the MCV
is normal.
Anemia-classification
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One way to classify anemia is by RBC size (i.e., MCV), as microcytic,
macrocytic, or normocytic. For the microcytic anemias, the etiologic
possibilities are iron deficiency, thalassemia, sideroblastic anemia, and
the anemias of chronic disease. Severe microcytic anemia (MCV <70 fL)
is caused mainly by iron deficiency or thalassemia. Macrocytic anemia
may be the result of megaloblastic (folate or vitamin B12 deficiency) or
nonmegaloblastic causes. Folate deficiency can in turn be due to either
reduced intake or diminished absorption. Severe macrocytic anemia
(MCV >125 fL) is almost always megaloblastic. In some rare cases,
macrocytic anemia is related to the myelodysplastic syndromes prior to
or after chemotherapy.
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The causes of normocytic anemias include aplastic anemia, bone-marrow
replacement, pure red-cell aplasia, anemias of chronic disease, hemolytic
anemia, and recent blood loss. A number of anemias have a genetic
etiology. Examples of such inherited disorders include hereditary
spherocytosis and sickle-cell (SC) anemia
Hypochromic Microcytic Anemia
D/D
Microcytic anemias
the etiologic possibilities are
 Iron deficiency
 Thalassemia
 Sideroblastic anemia
 Anemias of chronic disease.
Severe microcytic anemia (MCV <70 fL) is
caused mainly by iron deficiency or
thalassemia.
Normochromic Normocytic Anemia
D/D
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Chronic inflammatory disease—
(1)infection (2)collagen vascular disease
(3)inflammatory bowel disease
Recent blood loss
Malignancy/Marrow infiltration
Chronic renal failure
Transient erythroblastopenia of chidhood
Marrow aplasia/hypoplasia
HIV infection
Hemophagocytic syndrome
Macrocytic Anemia D/D
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Megaloblastic anemias
• Vit.B12 def. - (1) pernicious anemia
(2) malabsorption
• Folate def. - (1) malnutrition (2) malabsorption
(3) chronic hemolysis (4)drugs - phenytoin, sulfa
Hemolysis
Myelodysplastic syndrome
Marrow failure - Aplastic anemia
Chronic liver disease
Hypothyroidism
Macrocytic anemia D/D
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Macrocytic anemia may be the result of
megaloblastic (folate or vitamin B12
deficiency) or nonmegaloblastic causes.
Folate deficiency can in turn be due to either
reduced intake or diminished absorption.
Severe macrocytic anemia (MCV >125 fL) is
almost always megaloblastic.
The Normocytic Anemias
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These may be classified as follows:
underproduction of erythrocytes due to
(1) the anemia of chronic disease
(2) marrow failure
(3) renal failure (decreased erythropoietin)
loss or destruction of erythrocytes due to
(1) hemolysis
(2) acute blood loss
The reticulocyte count is useful in drawing this
distinction, being elevated in (b) and reduced in (a).
The Normocytic Anemias
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The causes of normocytic anemias include
aplastic anemia, bone-marrow replacement,
pure red-cell aplasia, anemias of chronic
disease, hemolytic anemia, and recent blood
loss. A number of anemias have a genetic
etiology. Examples of such inherited
disorders include hereditary spherocytosis,
sickle-cell (SC) anemia, and thalassemia
Hemolytic Disorders
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Hemoglobinopathy– Hb SS,SC,S-Bthal
Enzymopathy--G6PD def, PK def
Membranopathy—Hereditary spherocytosis,
elliptocytosis
Extrinsic factors—DIC, TTP, HUS,
Immune hemolytic anemia---Autoimmune, Drug
induced
Diagnostic Approach-History
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Age : Iron def rare without blood loss before 6
months in term infants.
Family Hist &Genetics:
(1)X-linked: G6PD deficiency
(2)Aut dominant: Spherocytosis
(3)Aut recessive:Sickle cell,Fanconi anemia
(4)Family member with early age of
cholecystectomy/splenectomy
(5)Ethnicity (Thalassemia: Mediterranean;
G6PD def: (Greeks, Blacks, Middle eastern)
(6)Race:B-thal:Mediterranean,African,Asian;
A-thal: Blacks, Asians
Diagnostic Approach-History
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Nutrition: (1)Cows milk diet:iron def.
(2)Strict vegetarian:Vit B12 def.
(3)Goats milk: Folate def.
(4)Pica: Plumbism,Iron def.
(5)Cholestasis, malabsorption:Vit E def
Drugs: (1)G6PD: oxidants (sulfa, primaquine)
(2)Immune mediated hemolysis(penicillin)
(3)Bone marrow suppression(chemotherapy)
(4)Phenytoin increases folate requirement
Diagnostic Approach-History
Diarrhoea-Malabsorption of VitB12/E/Fe.
Inflammatory bowel disease and anemia of
chronic disease with or without blood loss.
Milk protein intolerance induced blood loss
Intestinal resection: Vit B12 def
Infection: Giardia:iron malabsorption
Intestinal bacterial overgrowth:Vit B12 def
Fish tapeworm:Vit B12 def
-EBV,CMV,Parvovirus:BMsuppression
Mycoplasma,Malaria:hemolysis
Hepatitis:aplastic anemia
Endocarditis, HIV
Physical exam reveals presence
and potential causes of anemia
Fever-acute infection,intravascular
disease,collagen vascular disease
 Jaundice suggests hemolysis
 Petechia & Purpura—bleeding tendency
 Hypertension & edema-renal disease
 Hepatosplenomegaly and lymphadenopathy—
infiltrative disease
 Growth failure or poor wt. gain—Anemia of
chronic disease or organ failure
 Examine stool for blood; urine for hemoglobinuria
(Admit & tranfuse if signs of CHF)
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Physical Findings in Anemia
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Skin: Hyperpigmentation, café-au-lait spotsFanconi anemia
Petechia & purpura-BM infiltration, autoimmune
hemolysis & thrombocytopenia
Erythematous rash-Parvovirus, EB virus
Butterfly rash-SLE ; Vitiligo-Vit B12 def.
Head:Frontal bossing-Thalassemia major,
Microcephaly-Fanconi anemia
Mouth: Glossitis-B12 def, iron deficiency
Angular stomatitis-Iron deficiency
Pigmentation-Peutz Jeghers syndrome
Telangiectasia-Osler Weber Rendu syndrome
Physical Findings in Anemia
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Extremities:Absent thumb-Fanconi anemia
-Spoon nails-Iron deficiency
-Dystrophic nails-Dyskeratosis congenita
CNS-Irritable, apathy-Iron def.
-Peripheral neuropathy-lead poisoning
-Ataxia, post.column signs-Vit B12 def
-Stroke-Sickle cell anemia
Short stature-Fanconi anemia, Malnutrition
Laboratory Evaluation
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Hematology: Complete Blood Count, Retic count,
Peripheral smear, ESR, G6PD, Sickling(+/- inf),
Hb electrophoresis, Coombs Test, Osmotic fragility test,
BM aspiration
Biochemistry: LFT, UE, RFT, S.Ferrtin,
S.Haptoglobin, Iron, VitB12, Folate, Ceruloplasmin
Serology:Heterophil antibody, ANA,Viral
Urinalysis, microscopy, culture/sensitivity
Stool exam. for ova, parasites, occult blood
Endoscopy: upper and lower bowel
Imaging: US Abdomen, Skeletal radiographs, Tc
pertechnetate scan for Meckels diverticulum
Tissue biopsy: skin, lymph node, liver
Laboratory Evaluation
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Hematology: Complete Blood Count, Retic count,
Peripheral smear, ESR, G6PD, Sickling(+/- inf),
Hb electrophoresis, Coombs Test, Osmotic fragility test,
BM aspiration
Biochemistry: LFT, UE, RFT, S.Ferrtin,
S.Haptoglobin, Iron, VitB12, Folate, Ceruloplasmin
Serology:Heterophil antibody, ANA,Viral
Urinalysis, microscopy, culture/sensitivity
Stool exam. for ova, parasites, occult blood
Endoscopy: upper and lower bowel
Imaging: US Abdomen, Skeletal radiographs, Tc
pertechnetate scan for Meckels diverticulum
Tissue biopsy: skin, lymph node, liver
Microcytic Anemia
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TEST
S.Iron
S.Ferritin
Marrow iron
Hb A2 or F
Iron def ThalMin An of ch dis
- low
normal normal
- low
N/H
N/H
- low
N/H
N/H
- N
H=Bthal
N
N=Athal
RDW
- high
normal
N/H
MCV ÷ RBC - >13
< 13
Sickle/B-thal – Hb S > Hb A
Absence of microcytosis in both parents excludes B-thal
or Sickle/B-thal but not A-thal
Anemia-treatment
How can Anemia be treated ? As there are many factors causing
anemia, the main treatment is for the cause.
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Specific treatment of disorder causing anemia.
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Replacement of missing factors (iron, vitamin B12, folate)
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In many chronic anemias regular blood transfusions are needed
(thalassemia, myelodysplastic syndrome)
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Blood transfusion is usually needed in acute blood loss or if the
patient has severe symptoms or in heart failure
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(Problems of blood transfusion)
Iron deficiency anemia (IDA)
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It is a condition when supply of iron in the
body to bone marrow falls short of that
required for the production of red blood
cells. It is the commonest cause of anemia
throughout the world.
Iron Deficiency Anemia
The incidence of anemia in the general
population is about 1.5%.
 Iron deficiency related to inadequate
replacement of lost iron is the most
frequent cause of asymptomatic anemia
and has a variety of causes.
 Iron deficiency is common among
women of childbearing age; 10% to 20%
of menstruating women have
abnormally low concentrations of
hemoglobin (usually <12 g per 100 mL).
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Iron Deficiency Anemia
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What are the causes of IDA ?
Increased physiological demand for more red blood cells
eg: increased physical activity.
In children, during spurts of growth.
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In women during menstruation, pregnancy, parturition
and lactation.
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Inadequate dietary intake due to poor economic reasons
or deficient foods.
Decreased absorption due to disorders in the digestive
system.
GI blood loss Peptic ulcer, piles, hiatus hernia, carcinoma of
stomach, carcinoma colon, chronic ingestion of a certain type
of pain relievers, hookworm infestation.
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Iron Deficiency Anemia
Between 20% and 60% of pregnant
women have hemoglobin levels <11 g per
100 mL. Anemia was found in 6% of
white women and 17% of black women
during the first trimester and in 25% of
white women and 46% of black women
during the third trimester.
Iron Deficiency Anemia
The high rates of anemia in pregnant
women are not attributable to iron
deficiency alone, however. In a large study
of low-income, urban, mostly minority
pregnant women, only 12.5% were iron
deficient. Women progressing through
pregnancy develop a lowered hematocrit as
a result of physiologic hydremia related to a
disproportionate increase in plasma volume.
Iron Deficiency Anemia
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The daily oral iron requirement for men and
postmenopausal women is 0.5 and 1 mg
Menstruating and pregnant women have higher
requirements: 2 mg and 2.5 mg, respectively.
The replacement of lost iron is complicated by
the fact that only 5% to 10% of the 10 to 20 mg of
the iron in the average adult diet is absorbed.
Anemia is less likely to occur in women taking
birth control pills and more likely to occur in
women with intrauterine devices.
Iron Deficiency Anemia
Because men and postmenopausal
women rarely develop iron
deficiency that is not related to
gastrointestinal blood loss (often
occult), an evaluation of
gastrointestinal tract must be
performed when an iron deficiency
is detected in these individuals
IDA-Practice Point
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Iron def. is common in children 9mo-3yr
Iron def. anemia in a child over 3yr should
prompt consideration of occult blood loss.
Infants less than 6months generally do not
develop iron def.; the exception to this rule is
premature infants,who are at risk of iron def.at
4mo, if iron supplementation is not given.
Iron Deficiency Anemia-Diagnosis
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Red blood cell count may be normal or
decreased.
Peripheral blood smear shows pale small
cells.
Aniso-poikilocytosis
White blood cell count usually normal
Serum iron is reduced
Total iron binding capacity of blood shows
an increase.
Low serum ferritin
Iron Deficiency Anemia-Treatment
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Correction of iron deficiency - to
restore hemoglobin level.
To replenish iron stores.
Oral iron administration is advised.
(Side effects of oral iron)
Parenteral iron may be needed
occasionally
Treat the underlying cause
Anemia of Chronic Disease
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Patients with cancer, infection, or
inflammation, commonly have a mild-tomoderate anemia caused by red cell
underproduction. This 'anemia of chronic
disease' is very common, and is usually
normocytic. Some cases develop
abnormalities of iron metabolism, in which
case there may be a microcytosis.
Hemolysis
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(b)
(c)
(d)
Hemolysis is defined as the premature
destruction of red blood cells, from whatever
cause.
Recognising the presence of hemolysis
The simplest tests are
Raised reticulocyte count
Raised indirect (unconjugated) bilirubin
Raised serum LDH
Diminished serum haptoglobin concentration.
Further more sophisticated testing may be
needed in some cases.
Hemolysis
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A Direct Coombs' Test tells you whether the
red blood cells are antibody-coated, and, in
the presence of hemolysis, indicates an
immune-mediated process (Autoimmune
hemolytic anemia).
Note that many patients with a positive
Direct Coombs' test do not have hemolysis.
Hemolysis
Principal causes
Inherited abnormalities
* Membrane (Hereditary Spherocytosis)
* Hemoglobin (Sickle Cell Anemia)
* Enzymes (Glucose-6-phosphate
dehydrogenase (G6PD) deficiency)
Hemolysis
Acquired causes
 Immune
Warm and Cold Autoimmune Hemolytic
Anemia
 Non-immune
* Mechanical Damage from leaky heart valves
* Microangiopathic hemolytic anemia
(MAHA) like TTP, HUS & DIC
Lead Poisoning
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Hypochromic microcytic anemia
Associated iron deficiency
Child has pica and is exposed to lead paint or
lead dust
Blood smear shows basophilic stipling and
blood lead is elevated.
Removal from exposure,chelation therapy and
correction of iron deficiency are important.
THALASSEMIA
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Normal Hb is a tetramer of 2 alpha and 2 beta
chains
Alpha-thalassemia:decrease or total lack of alpha
globin synthesis
Beta-thalassemia:decrease or total lack of beta
globin synthesis
THALASSEMIA
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Clinical classificationSilent carrier(AorB):normal CBC
Thal trait(AorB):mild anemia(HM)
HbHdisease(A-thal):moderately severe hemolytic
anemia,icterus,splenomegaly
Severe Beta-thal:severe anemia,growth
retardation,hepatosplenomegaly,bony def.
Thalassemia major:tranfusion dependent
Thal-intermedia:no regular transfusions
THALASSEMIA-complications
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HbH disease:severe hemolytic
anemia,spenomegaly,hypersplenism
Thal-major:poorly trasfused-skeletal
abnormalities,growth retardation,CHF
Thal-major:well transfused with iron overload(1)Endocrine disturbances:delayed puberty,growth
retardation,diabetes mellitus,hypothyroidism
(2)Cardiac:arrhythmias, congestive heart failure
(3)Hepatic:cirrhosis,liver failure
THALASSEMIA-Lab
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Thal trait:Hb 9-10 g/dl
HbH disease:Hb 6-7 g/dl
Thal intermedia:Hb 7-8 g/dl
Thal major:Hb less than 5 g/dl
Peripheral smear:hypochromic,microcytic,
anisopoikilocytosis,target cells
Hb electrophoresis: (1)Thal trait-HbF 1-5%, HbA2
3.5-8%,rest HbA
(2)Thal major- HbF 20100%,HbA2 2-7%,HbA 0-60%
THALASSEMIA-therapy
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Red cell transfusion 3-4 weekly-Hb 9-10
Chelation therapy with desferrioxamine
Splenectomy if transfusion >200ml/kg/yr
Folic acid 5mg daily
Penicillin prophylaxis to all splenectomised
Pneumococcal and Hib vaccine before sply.
Cholecystectomy for gall stones
Bone marrow transplantation is curative
Genetic counselling
SICKLE CELL DISEASE
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SA :Sickle cell trait-asymptomatic
SS :Sickle cell anemia
S-Bthal:Sickle cell-beta thal
SC :Hb SC disease
Pathophysiology:Valine replaced by
glutamic
acid at Beta 6 position.
With
deoxygenation HbS crystallises&gels
Clinical features
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Anemia:chronic,onset at 3-4 mo
Aplastic crisis:parvo virus B12
Sequestation crisis:usuallyspleen
Hemolytic crisis
Dactylitis:Hand foot syndrome(infant)
Painful crisis:muscle,bone,bone marrow,lung, intestines
Cerebrovascular accidents
Acute chest syndrome:infection,infarction,emb
Chronic lung disease:pulmonary fibrosis,restictive lung
disease,cor pulmonale
Clinical features
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Priapism
Ocular:retinopathy
Gall bladder disease:stones,cholecystitis
Renal:hematuria,conc.deficit,nephropathy
Cardiomyopathy
Skeletal:avascular necrosis of femoral head
Leg ulceration:in older pts
Infections:pneumococcal pneumonia,meningitis,
arthritis,Hinf sepsis,salmonella&staph osteomyelitis,mycoplasma
pneumonia,viral infe
Growth failure,delayed puberty
Psychologic problems:chronic illness,chronic pain
THERAPY
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Anemia is usually chronic&compensated
Blood transfusion only given based on clinical condition,Hb
level&retic count
Crisis:Splenic sequestration crisis,aplastic crisis,hyperhemolytic
crisis-in all of these PRBC is indicated when anemia is sympto.
Pain crisis:IVF,analgesia with narcotics,NSAIDs
Acute chest syndrome:O2,judicial use of
analgesics&fluids,antibiotics,PRBC
Stroke:O2,fluids,exchange transfusion
Hydroxyurea:decrease number&severity of VOC
Bone marrow transplantation
G6PD deficiency
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Episodic hemolysis on exposure to oxidants
Severity of hemolysis depends on the
enzyme
variant
Gene for G6PD is on X chromosome
Jaundice,dark urine(bilirubin,hemoglobin),
Red cells appear blistered
G6PD levels may be normal with hemolysis
Therapy:PRBC,IVF,urine alkalinisation
Prevention:avoid oxidants,fava beans,henna