هذا ملخص علم الدم ستجدون به الثاليثيميا أحد ملخصات أخونا شادي جزاه الله خيرا
هذا ملخص علم الدم ستجدون به الثاليثيميا أحد ملخصات أخونا شادي جزاه الله خيرا هو و من ساعده فيها هذا هو الموقع لباقي الملخصات العشرة لأن ملخص علم الدم يمثل ملخص واحد منهم و شكرا :
بسم الله الرحمن الرحيم
طبعا الملخص اعتمد على المرجع الاساسي للهيئة السعودية للتخصصات الطبية .. (( يعني الملخص معتمد بإذن الله ))
طبعا هذي صورة الكتاب الذي تم استخدامه في كتابة الملخص ..(( وهو المرجع الذي تستخدمه الهيئة في وضع اسئلتها لقسم علم الدم ))
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*Hematopoiesis : Formation of different blood cells (( RBCs-WBCs-PLATELATE)) - Site : Red Bone Marrow in all bone of children , But in adult Restricted To Central Skeleton (( skull , sternum . ribs , hips , vertebra ))
* Factors Necessary For Erythropoiesis (( Formation of RBCs)) :-
1- erythropoietin hormone . 2- Iron
3- Vit B12 4- Folic Acid
5- Ascorbic Acid ( Vit C ) 6- Vit B6 ( Pyridoxine )
7- Amino Acid .
* RBCs : Red color , anucleated , biconcave shape .
Function of RBCs : - *
Transport Oxygen + CO2 + Hormone + Enzyme +Vitamins + Nutrients
Erythrocytosis ( Increase in red Blood Cells with physiological causes ) :-*
1- High altitudes 2- Physical training
3- Drugs 4- Smokers
*Polycythemia ( increase in red Blood Cells With Pathological causes ) :-
The Increase Of RBCs is Not By physiological causes .
*Erythrocytopenia :- ( Decrease in Red Blood Cells )
1- RBCs Loss : Hemorrhage 2- Abnormal destruction of red blood cells
3- lack substance needed for RBCs Production .
4- chemotherapy or radiation
5- Leukemia
* About 1% of RBCs per day are Breakdown
* Components of RBCs Are Recycled :-
Iron ----- Transfrrin ------} To bone Marrow
Amion acid -à globin
Most Urobilinogen are excreted in Stool , Some of it excreted in urine .
* Alteration in size of RBCs :- anisocytosis
* Alteration in shape of RBCs : Poikilocytosis
*Hemoglobin : Consist Of 4 Protein + 4 Heme Groups
( 94 % ) ( 6% )
+ = Iron (Fe) Bond
*Leucocytosis
Female : less than 20 mm\hr
:- increased in WBCs count
Neutrophilia (( neutrophil leucocytosis)) :- increased in neutrophil count * Causes :- -physiologic :
1-Pregnancy
2- after exercise 3- lactation . 4- neonates (( new borns )) -pathological :
1- acute pyogenic infection (( Bacterial ))
2- hemorrhage or hemolysis
3- truma or surgery
4- chronic myeloid leukemia
5- polycythemia rubra vira
6- Dohle bodies seen (( it is 1-2 um long – pale – grayish – blue cytoplasmic inclusions which are situated in peripheral of cells )) .
* Causes of anemia :
1- Decreased bone marrow production of RBCs : e.g Aplastic Anemia
2- Increased RBCs Loss : Hemolysis or Hemorrhage
3- Splenomegaly or hypersplenism
(A- Microcytic Anemia ) 1- Fe Deficeincy anemia : (( Commonest anemia in world ))
1-iron deficiency develop slowly
2- symptoms only seen when HB falls below 8 g/dl Causes :
a- Decreased intake : e.g Poverty
b- Increased requirement : pregnancy – lactation .
c- blood loss : GIT or Uterine
Diagnosis of fe deficiency anemia :
1- Microcytic ( MCV Less than 80 )
2- Hypochromic ( MCHC less than 27 ) is normal until later stage it is reduced .
3- High RDW (( more than 15% ))
4- Low Serum Fe
5- high Transferrin (( iron transport protein ))
6- Low Serum Ferrrtin (( method of choice for diagnosis ))
7- Low Bone Marrow Fe (( absent ))
8- anisocytosis + poikilocytosis .
9- pencil cells shaped (( elongated )) + may target cells
10- erythropoiesis is normoblast (( normal bone marrow production ))
2- Sidroblastic anemia : - is abnormal type of red cells production in which erythroblast contain perinucluear ring contain iron granules .
Diagnosis of Sidroblastic anemia :
1- Microcytic ( MCV Less than 80 )
2- Hypochromic ( MCHC less than 27 )
3- Bone mmarrow : Ringed Sidroblasts
Thalassemia
Diagnosis of Thalasemia :
1- Microcytic ( MCV Less than 80 )
2- Hypochromic ( MCH less than 27 )
3- Defective in Hb F (( more than 70% in Hb electrophoresis test ))
4- RBCs Has Neucluos ( normobalst )
5- Target cells
6- Absence of HbA .
7- Serum Iron High .
8- serum ferrtin High
((like the statue of one of family of sons of demon (( sami )) ))
(B- Macrocytic Anemia )
1- Megaloblastic anemia : abnormal re cell precursors known as " megaloblast" which cell and nuclear diameter are longer than normal .
it is Pancytopenia* Due To Deficiecy of Vitamin B12 or Folic Acid Or Both
* pancytopenia is : Decreased Count of RBCs And WBCs and Plateletes
Function of Vitamin B12 And Folic Acid :
It is important for Formation of DNA & RBCs .
Deficiency of Vitamin B12 And Folic Acid cause :
-Defective in DNA Synthesis ------} Defective in Hematopoiesis ----} pancytopenia
Normal amount vit B12 = 5 ug\day
Vit B12 store mainly in liver . Diagnosis of Megaloblastic Anemia :
1- Macrocytic anemia (( MCV more than 80 ))
2- Hyperchromic ( MCH more than 32 )
3- pancytopenia :
- anemia : low Hb , Low RBCs , Low Hct
- Leucopenia : Low WBCs
- Thrombocytopenia : Low Platelets
4- Decreased Serum Level of B12 Or Folic Acid .
5- Abnormal in Bone Marrow Cells :
- Abnormal Erythroid Cell ( RBCs )
- Abnormal Myeloid Cell ( WBCs )
- Abnormal megakaryocytes cells ( Platelets )
6- horse shoe shaped cell .
7- variable reticulocyte count (( normal – increased – decreased ))
8- reduce red cell life span .
9- oval shaped cell
10 – anisocytosis + poikilocytois .
( C- Normocytic anemia )
1- Aplastic Anemia ( AA) : it is pancytopenia Due to Bone Marrow failure
Causes :-
A- congenital : fancoin's anemia
B- acquired :
1- Drugs : chloramphenicol = in long time ) and (cytotoxic drug )
2- Chemical : Benzene
3- Radiation .
4- viruses hepatitis C )
* diagnosis of aplastic anemia :
1- normocytic ( MCV is normal 80-100) or macrocytic .
2- normochromic ( MCHC normal 27-32 )
3- pancytopenia
4- Bone marrow failure ( decreased in bone marrow cell or no bone marrow cells)(( hypocellular bone marrow ))
5- low reticulocyte count
6- marked increased of erythropoietin level in serum and urine
7- Enlargement of spleen .
8- increased in serum erythropoietin .
9- markedly decreased amount hematopoitic tissue in bone marrow .
10- fancoin's anemia is disease of aplastic anemia .
Pure red cell aplasia ;-
affect only the erythropoietin cells
patient with aplasia have anaemia + reticuloculocytopenia with normal WBC and platelate count
Pernicious anemia :-
It is megaloblastic anemia due to Vit B12 deficiency .
Diagnosis by schilling test .
-there are failure in absorption of Vit B12 due to Marked reduction on intrinsic factor secretion in stomach .
* Diagnosis of Pernicious anemia :-
1- low serum B12 .
2- low Hb .
3- Macrosytic (( high MCV ))
4- hematopoiesis is megalobalst (( abnormal bone marrow production ))
5- high serum billirubin .
6- high LDH (( lactate dehydrogenise ))
7- normal folate or may low
8- marked reduction or absence of intrinsic factors in gastric juice (( Deteted by schilling test )) .
anaemia of chronic disorders :
common type of anaemia seen in hospital in patient with chronic disorders such as (tuber culosis ) & (rheumatic arthritis )
diagnosis :
under production of red cells (decrease erythrocytosis) .
reduction of red cell life span (hypersplenism ).
most normocytic – normochromic .
30 -35 % microcytic – hypochromic.
low serum iron (fe).
high serum ferrtin .
low serum transferrin .
increase bone marrow fe .
(( Hemolytic Anemia ))
It is anemia due to shortend RBCs Life Span . (( cause of hypersplenism ))
B- Outside RBCs ( Extracorpuscular ) :
a- Immune Hemolytic Anemia: IGg responsible for it , E.g : SLE
b- Non – Immune Hemolytic Anemia : E.g : Hypersplensim :: reduction in the life of red cells .
Diagnosis of Hemolytic anemia :
1- increased Indirect Bilirubin ( Jaundice )
2- Reticulocytosis ( Due to erythroid cell in bone marrow )
3- decreased Haptoglobin ( Hemoglobin binding Protien )
4- shortend RBCs Life Span .
- G6PD deficiency ---} low rate of bleeding time .
- G6PD deficiency ---} cause seen Heinz bodies .
- G6PD deficiency ---} normochromic anemia .
Hemolytic Anemia
-Abnormal red cells are removed from circulation by phagocytosis from macrophage in spleen , liver , bone marrow .
-diseases of hemolytic anemia are :
1- hereditary spherocytosis 2- hereditary elliptocytosis
3- Glucose 6 phosphate dehydrogenase ( G6PD ) deficiency . 4- Sickle Cell Anemia 5- Thalasemia 6- acquired hemolytic anemia .
*Laboratory diagnosis of hemolytic anemia :
1- increased red cell destruction .
2- increased erythropoitic activity .
*laboratory evidence of increased red cell destruction :-
- simple method to detect red cell destruction by estimating the amount of unconjugated ( indirect ) bilirubin in plasma .
-Free hemoglobin in plasma called ( hemoglobinemia ) .
-when hemoglobinemia is present , some of free Hb converts to dimers and dimer pass through the kidney causing hemoglobinuria ( hemoglobin in urine ) .
Some of dimer taken by renal tubular cells and convert to hemosiderin .
-Hemosiderin detected by perl's acid Ferro cyanide reaction { Perl's Prussian blue}.
-Hemosiderin is golden – brown and blue ganules when stain by perls .
*Morphology of red cell in hemolytic anemia blood film :
-cerful examination of blood film may indicate the occurrence hemolysis by presence of damaged or abnormal cells shapes :
1- Microspherocyte .
2- red cell fragments (( schistocyte )) * important
3- sickle red cell .
4- cell containing malaria parasite .
laboratory evidence of increased erythropoitic activity :-
reticulocytosis .
erythroid hyperplasia
- increased of erythroblasts ( nucleated red cell ) in the circulation associated with erythroid hyperplasia .
*Relation between erythroid hyperplasia and reduced M/E ratio :-
-M/E ratio is : ratio between number of cell of neutrophile series and the number of erythroblast in the bone marrow .
- Normal range : 2:8
- reduction of M/E ratio is taken as evidence of erythroid hyperplasia .
G6PD Deficiency :-
Type B is common type , and the defective gene is present on the chromosome X , Minaly seen in males .
FAVISM :- is acute hemolytic anemia result from G6PD Deficiency , usually effect in children .
* Tests Fot Diagnosis Of Sickle Cell Anemia :
1- Sickiling test .
2- Hb electrophoresis (( Hb A , Hb S , Hb F )) .
-------------------- Systemic Lupus Erythematosis ( SLE ) :
It is Autoimmune Disease affecingt many Organs . *Tests of SLE :
- by Detection of anti-body ( Abs ) :
1- antinuclear Ab ( ANA ) : But not
specific test for SLE
2- Anti-Double Stranded- DNA Ab ( AdsDNA )
: it is specific test for SLE
* diagnosis of SLE : - Blood Analysis :
1- pancytopenia 2- high ESR - Urine analysis
1- proteinuria ( protein in urine )
2- hematuria ( blood in urine )
3- RBCs & WBCs Casts .
THERE ARE 2 SYSTEM FOR CLOTTING TEST ;
partial thromboplastin time ((PTT))
prothrombin time ((PT))
thrombin time ((TT))
THROMBIN TIME :- is prolonged when there is deficiency of fibrinogen and presence of heparin .
2- Partial thromboplastin time ((PTT)) ;
it is measure intrinsic system
consist of six factor (XII-XI-IX-X-II-I)
it is prolonged when there is deficiency of factor ((VIII-IX))
when VIII deficiency it causes ; a- hemophilia &hemophilia A. b- Von willebrand disease .
when IX deficiency it causes; a-hemophillia B. b- Christmas disease
we measure PTT by adding ;-
a- kaolin , b- phosphlipid . c - calcium.
3- PROTHROMBIN TIME (PT) :
- it is measure extrinsic system and consist of two factor :
a- I I I ; TISSUE THROMBOBLAST
b- VII
- it is prolonged when deficiency of (( I , II , V , VII , X ))
- sensitive special to deficiency of (( V , VII , X ))
- we measure PT by adding ; a- thromboplastin . b- calcium .
THERE IS SIX FACTOR SHOULD BE KNOWN ;
VIII :- anti-hemophillic
IX :- Christmas
II :- prothrombin
IIA :- thrombine
I :- fibrinogen
IA :- fibrin
Some convert of factor :
plasminogen convert to plasmin by : a- kallikarin . b- urokinase . c- streptokinase .
fibrin convert to fibrin degradation product by : plasmin .
fibrinogen to fibrin by : thrombin .
Anticoagulant drugs : most two drug are :
1- Heparin . 2- Warfain
Heparin :- it is acidic mucopolysaccaride
it is potentiates action of anti thrombin III .
Heparin does not cross placenta .
Hemorrhage due overdose of heparin manged by ;
Stopping heparin by giving protamine sulphate .
Warfarin :- it is vitamin K antagonist .
Warfarin cross placenta .
Hemorrhage stopping by :
Stop Warfarin and infusing fresh frozen plasma and give vit K
ANTI THROMBIN III : it is mainly inhibitor of thrombin and also factors (( IXA – XA – XIA ))
it is potentiated by Heparin .
protein C and protein S :
protein C is protease and degrades factor ((VA – VIIIA ))
protein S is potentiated and effect of activated protein C .
THE TWO ARE VITAMIN K DEPENDANT SUBATANCE .
PLATELATE :-
Discoid , non nucleated &granules .
Formed in the bone marrow by megakaryocytic
Normal range 160000-450000/L
Life span is 10 days .
Main function is formation haemoatatic plug at the site of damage
Bleeding time :
normal 2-4 min with IVY method
this test depend on platelate and capillary fragility .
Leukemia
are neoplasms of the precursors of blood cell are characterized by accumulation of abnormal WBC in bone marrow .
DIAGNOSIS OF ACUTE LEUKEMIA ;
Normochromic + normocytic anaemia .
Thrombocytopenia (( common))
WBC high ((increased ))
Neutrophil count decreased(( markedly))
Lymphoblast small in ALL .
Hypercellular bone marrow
Ph chromosome in their blast cell in ALL .
DIAGNOSIS OF CHRONIC LEUKEMIA ;
Normochromic + normocytic
Ph chromosome found
WBC high
Thrombocytosis ((high platelets ))
Basophill and eosinophil and monocyte increased
Neutrophil alkaline phosphatase decreased
Vitamin B12 increased
Hyperplasia of neutrophil , eosinophil basophil (granules )
Polycythemia : it is increased RBCs count , Hb , Hct , high PCV
Types of it :
1- primary polycythemia.
2- Secondary polycythemia.
3-Relative Polycythemia.
-Relative Polycythemia : is due to decreased plasma volume with normal RBCs count.
– relative increased of PCV but normalWBCs and Platelets
e.g : Burns , diuretic ttt
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