ANAEMIA


ANAEMIA
HEMOGLUBIN NORMAL LEVEL:
MEN= 14 – 17.7
WOMEM= 12 - 16
This disease is present when the level of hemoglobin and red cells in the blood are decreased below the reference range for the sex and age of the individual.
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SYMPTOMS:
Clinical symptoms depend on speed of onset of blood lose and anemia.
Example:
Hemoglobin slowly falling level will be compensated by the organism.
Young patients can tolerate anemia more than elders.
Anemia can cause angina pectoris – fatigue – breathlessness – intermittent claudication – cardiac failure in elderly people.
CLASSIFICATION
        OF ANEMIA:
A-MICROCYTIC ANEMIA: MICROCYTIC.
First:
Iron deficiency:
The main thing can cause a microcytic anemia is iron deficiency.
In the duodenum and jejunum where iron is absorbed and transported in the plasma and bound with transferring.
1mg of iron everyday is lost in urine-sweat and faeces.
Women lose iron during premenopausal and menses.
In general the main cause of the iron deficiency is blood lose from the gastrointestinal tract in general and from the uterus in females during growth and pregnancy.  
Other reasons of iron deficiency:
Small bowel disease.
Poor intake.
Symptoms:
Dysphagia - angular stomatitis - hair lose-spoon shaped nails.

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Investigations:
Blood account:
Low hemoglobin Hb and MCV.
Blood film:
Microcytic and hypochrmic.
Blood serum:
Iron is low – iron bending capacity is high.
Ferrtitin is low which reflects iron stores in organism.
Treatment:
Treat the main cause.
Oral iron tablets for six months.
Parenteral iron used for intolerant patient or poor response oral iron.

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SECOUND:
Talassaemia:

Third: Sidroblastic

Fourth:
Anemia of chronic disease:
Cause decreasing release of iron from the boon marrow for developing erythroblasts and decreasing red cell survival.

Different chronic disease cause this kind of anemia/ normocytic or microcytic anemia/
And need to be differentiated from iron deficiency.
By iron laboratory findings.

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Chronic diseases are:
Chronic infections.
CRF. Chronic renal failure.
Inflammatory disease.
Malignancy.
                                  …..
 Treatment:
Controlling and treating the underlying disease.

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MACROCYTIC ANIMEA
HIGH
MCV:
The peripheral red cells are large.
A group of anemias the erythroblasts present in the boon marrow with delayed nuclear maturation because of defective DNA synthesis that could also affect the white cells leads to hypersegmentd neutrophils and leucopenia.
The most common reasone: vitamin
B 9-12 DEFICIENCY
Both of them necessary for DNA syntheses
Vitamin B12 deficiency:
Vitamin B12 is obtained from fish-red and other meats- milk - eggs …. from animal sources.
B12 is formed from protein complexes in food and binds to one factor that secreted from parietal cells in stomach.
B12 is absorbed in terminal ileum and transported to organism by transcobalamin I - II
CARRIER PROTEINS.
B12 is stored in the liver and is sufficient supply for two years.

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CAUSES OF VITAMIN
B12 DEFICIENCY:

Low B12 intake.
Vegetarian.
Impaired absorption:
Pernicious anemia.
Gastrectomy.
Small bowel disease and malabsorption:
Tropical spure.
Celiac disease.
Infection overgrowth.
Pancreatic disorder.
 Chronic pancreatitis.
……………………….
One of the reason of B12 deficiency is pernicious anemia
What is this kind of anemia?
What age has affected?
And what is the pathology?
It is an autoimmune disease associated with atrophy of gastric mucosa and failure of intrinsic factor production.  
 This disease affects elderly people over the age of sixty years old and it's common more in women with fair hair and blue eyes.
occurs with other autoimmune disease association like hypothyroidism.

The pathological reason of pernicious anemia is the sever atrophic gastritis that lead to absent of gastric acid and intrinsic factor secretion.

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SYMPTOMS OF PERNICIOUS ANEMIA:
This kind of anemia is insidious, occurs with progressively increasing symptoms of anemia.
Could be glossitis – angular stomatitis – jaundice….
Neurological symptoms of pernicious anemia:
Dementia – optic atrophy –
peripheral neuropathy:
progressive weakness
Ataxia – Paraplegia because of sub acute degeneration of demyelintion of the corticospinal tracts and posterior columns.

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Laboratory 
investigations:
Blood count and film:
Hyper segmented neutrophils.
Leucopenia and thrombocytopenia occur in very severe cases.
Serum B12 vitamin:
Is low. Normal > 160 ng/L.
Red cell folate: reduced.
Serum bilirubin:
Is raised caused of ineffective erythropoiesis in the bone marrow witch lead to excess breakdown of hemoglobin.
Serum auto antibodies:
90% of cases present parietal cell antibodies.
50% of cases present intrinsic factor antibodies.
SCHILLING TEST:
Very important for differentiating penicious anemia from malabsorption anemia as the cause of B12 deficiency.
BONE MARROW EXAMINATION:
Hyper cellular bone marrow with megaloblastic changes…

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COMPLECATION OF PERNICIOUS ANEMIA:
Gastric carcinoma.
MANAGEMENT:
Intramuscular injection 1mg of hydroxycobalamin twice per week for three weeks.
Then three times per month continued for life.    
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Folate deficiency anemia:
Folic acid is found in green vegetables and offal.
The absorption process of folate goes in the upper part of small intestine.
Anemia caused by folate deficiency could be the result of poor dietary intake of vegetables.
This kind of anemia commonly occurs with malabsorption or increased folic acid utilization.

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Physiological causes of folic acid deficiency:
Pregnancy.
Lactation.
Prematurity.
Pathological causes of folate deficiency:
Inflammatory diseases:
Ex. Tuberculosis – psoriasis – crohn disease…..
Malabsorption:
Tropical spure.
Celiac disease.
Drugs:
Trimthoprim.
Phenytoin.
Other reason of folate deficiency:
Alcohol excess – anorexia – old ages – poverty.
SYMPTOMS:
All symptoms appear because of anemia.

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Laboratory investigation:

Serum folate is low.
Normal level:
< 3 - 15 >
Red cell folate is also low.
Normal level:
< 160 – 640 >
Note:
If there is no dietary poor intake of folic acid then should perform the jejuna biopsy investigation to look for small bowel disease.

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DIFFIRETIONAL DIAGNOSIS:
Macrocytosis with MCV on the film of peripheral blood occur with a normoblastic rather than a megaloblastic bone marrow.
FLOIC ACID DIFICIENCY
TREATMENT:
By giving 5mg folic acid/day.
Treating underlying cause.
Folate prophylactic treatment:
In pregnancy and patients with chronic haemolysis.


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MARROW FAILURE
APLASTIC ANEMIA:

It is pancytopnia with hypocelluarity of the bone morrow means reduce of all blood count cell. It's very serious condition could leads to death.

CAUSES OF APLACTIC ANENIA:

Congenital – chemicals – drugs – radiation – infections.
Infiltration of bone marrow: like:
Acute leukemia – myeloma – lymphoma – carcinoma.
Abnormal bone marrow maturation:
Ex.
Myelodysplasia.
Megaloblastic anemia.
Excess destruction: as in hypersplenism.
Auto immune disease:
 witch cause antibody formation like in lupus erythematosus.
APLASTIC ANEMIA MANGEMENT:
The treatment course of aplastic anemia is very variable and ranged between different stages. 
Need to eliminate the  main cause of this kind of anemia.
Transfusion of red cells platelets.
Antibiotic therapy.
Bone marrow transplantation.
Immune suppressive therapy.
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HAEMOLYTIC ANEMIA
It's caused of increasing destruction of red cells reducing of lifespan.
NORMAL RED CELLS LIFE TIME IS 120 DAYS.
INHERTED CAUSES OF HAEMOLIC ANEMIA:
HEREDITRY SPHEROCYTOSIS.
HEREDITARY ELLIPTOCYTOSIS.
ABNORMALITIES OF HAEMGLOBINE:
THALASSAEMIA.
SICKLE-CELL DISEASE.
METABOLIC DEFCTS:
GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENY.
PYRUVATE KINASE DEFICIECY.
ACQUIRED CASUSES HAEMOLTIC ANEMIA:
IMMUNE SYSTEM DISORDER:
Autoimmune  hemolytic anemia.
Hemolytic transfusion reactions.
Miscellaneous:
Infection.
Chemicals – drugs.
Hypersplenism.
 OTHER RESEONE OF HEMOLTIC ANEMIA:
March hemoglobinuria.
Paroxysmal nocturnal hemoglobinuria.
Microangiopathic hemolytic anemia.
MACROPHAGES IN THE RETICULOENDOTHEL-IAL SYSTEM DESTRUCTED RED CELLS FROM THE CIRCULATION.
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INHERITED HAEMOLTIC ANEMIA:
SPHEROCYTOSIS:
It's heredity disease most commune inherited in northern Europeans.
Caused by deficiency of one protein called spectrin, this protein very important for red cell membrane formation.
Spectrin deficiency leads to deformation of red cell shape, which become spherical in shape these cells are more rigid and they are less deformable than normal red sell.
That pathological shape lead to be destroyed by spleen prematurely.

SYMPTOMS OF HEREDITARY SPHEROCYTOSIS ANEMIA:
Jaundice – symptoms of anemia – splenomegaly – legs ulcers.
CHROCNI HEMOLYSIS LEADS TO PIGMENT GALLSTONES FROMATINS.
LABORATORY FINDINGS:
Blood count:
Mild anemia with reticulocytosis.
Blood film:
Spherocytes and reticulocytes.
Diagnosis made by:
Increase red sell osmotic fragility when placed in hypotonic solution.
                
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SPHEROCYTOSIS ANEMIA MANAGEMENT:
In all cases should perform splenoctamy and all patient shoul resive pneumococcal vaccine with long term penicillin also for prophylactcs.



HEMOGLOBIN ABNORMALITIES
:
Normal HB made up of haem and two  polypeptide globin chains, Alpha and Beta chain.
Classifications of hemoglobin abnormalities:
I - Abnormal chain production.
II – Abnormal chain structure.
THALASSEMIA:
Normal hemoglobin has the balance 1:1 in production of Alpha and Beta chain.
Talassaemias have one or multiple gene defects that lead to reduce the production of one or more globin chain.

The production of imbalanced globin chain leads to precipitation of globin chain inside red cells. All that results in ineffective erythropoiesis and hemolysis.

TYPES OF THALASSEMIA:
I – a-Talassaemia with reduced a-chain synthesis.
II – b-Talassaemia with reduced a-chain synthesis.
B-THALASSEMIA:
I – Trait B-Talassaemia:
Asymptomatic stat.
Here stores of iron are in normal situation.
II – Intermia B-Talassaemia:
Moderate anemia and does not require regular blood transfusion.
Signs and symptoms:
Splenomegaly – leg ulcers – bone deformation – gallstones.
   III – major b-Talassaemia:
Present in the first year of the life with:
Recurrent  infections and severe anemia – hepatosplenomegaly.
Thalassaemic face:
Enlarged maxilla- prominent frontal and parietal bones.

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LAB. INVESTIGATION OF THALASSEMIA:
FILM AND BLOOD COUNT:
SHOW HUPOCHROMIC/MICROCYTIC ANEMIA.
Reticulocytes count and nucleated red cells in the peripheral  circulation are raised.
DIAGNOSIS :
Is made by the hemoglobin electrophoresis showing an increase of HB-F And reducing of HB-A.
TREATMENT AND MANGENENT:
I - Blood transfusion. That leads to
Iron overload and damaging the endocrine glands liver, heart => cardiac failure and pancreases.
II - Iron chelating agent.
III – Bone marrow transplantation in some cases.





    





 


Internal medicine.
Dr.Zafer Maklad
zafernm@yahoo.com


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