Lap midicine Flashcards
(45 cards)
General Features Of Hemolytic Anemias 8
1- Increased rate of red cell destruction, 2- Erythroid hyperplasia within the bone marrow → Reticulocytosis 3- Hemolytic jaundice: heme→ bilirubin → indirect or unconjugated
hyperbilirubinemia produces jaundice if >2.5 mg/dL
4- Pigment stones in gall bladder (? ↑ bilirubin in bile + long-standing hemolysis) → calcium bilirubinate gallstones
5- Generalized hemosiderosis or in severe cases, 2ndry hemochromatosis
due to excess iron accumulation 6- Extramedullary hematopoiesis in the spleen and liver of infants 7- Enlarged liver and spleen (splenomegaly) due to hyperplasia of the
mononuclear phagocyte system and extramedullary hematopoiesis.
8- Decreased serum haptoglobin (intravascular hemolysis → binding Hb →
hemoglobin-haptoglobin complex in the blood →Removed by macrophages →Markedly reduced haptoglobin levels Increased serum lactate dehydrogenase (LDH) from hemolyzed RBCs
The master iron regulatory hormone
Hepcidine
The master iron regulatory hormone
Hepcidine
Give me example Function of Hepicidine
Less transferrin iron less decrease Hepcidin so in the liver it is allowed to circulation ferroportine 1 to bind transferin
Hereditary spherocytosis
Defenation and etiology ,pathogensis
Autosomal dominant trait 25%Autosomal recessive
It is defect in the red cell membrane protine spectrin
Becomes spherocyte ➡️Less deformable destroyed by spleen by mQ= Extravascular hemolysis .
Lap finding of spherocytosis :
Decrease Hb ,Normal MCH with increase MCHC
Normocytic normochromic
Blood film :Spheroidal in shape with absence of the central zone of pallor pallor
General findings ⬆️Reticulcytic count ⬆️serum indirect bilirubin⬆️urine urobilinogen ,Serum haptoglubin normal
Special test increase osomatic fragility
Clinical finding of Sperocytosis treatment
Anemia ,
Jundence , increase pigment stone ,Splenomegaly ,In long standing cases systemic hemosidrosis
Treatment:Splenoctomy
Sickle cell Disease Defenation and etiology
Hereditary presence of abnormal Hb
Replacement glutamic acid by valine at the sixth position
Type of sickle cell disease
Homozygous state SS both gene is abnormal
Heterozygous is sickle cell trait AS
Phathogensis of sickle cell
First mechanism : The sickle cell is removed by phagocytosis
Second mechanism :Upon deoxygenating Hbs crystallization rigid crescentic bout like shape
Reoxygenation unsickling
Micro vascular occlusion ➡️pain crises
Clinical of Sickle cell anemia
Homozygone after six months
🩸splenomegaly in children 👦 but in Adult autosplenmegaly
Sudden vasocclusive or pain crises
Increase susceptialy of infection
The acute chest syndrome to death
Lap finding of sickle cell
1- General : normocytic ,normochromic decrease Hb Ht
2-General finding of Hemolysis :Marked reticulocytosis ,uncontrolled hyperbilirubinemia
3- Sickle cell disease =Irreversible ,Sickle cell trait =Normal
4- sickling test : sodium metabisufiter
Thalassemia Defenation and etiology
Caused by mutation decrease rate of synthesis of a or B globin chains
Four gene الفاً قلوبين 16
Tow gene بيتا قلوبين 11
الفاً thalassemia
Clinical disease
Four a-globin gene 🧬 on chromosome 16p
1- Silent -carrier state 75%
2- aThalassemia 50% production as mild Anemia Two of a is deleted microcytic hypochromic
3-Three of the four a-globin gene are deleted 25%a chaine
4- Hydrops fetalis Hb Bart Y4 ➡️No a chain fatal condition Lethal in utero
B - thalassemia Clinical disease state
Autosomal recessive
1- B-Thalassemia minor ➡️Asymptomatic Milde hypochromic microcytic anemia
2- B-Thalassemia intermedia ➡️sever hypochromic microcytic anemia
3- B-thalassemia Cooley’s insoluble aggregate
Regular blood transfusion required
Clinical in B thalassemia
1- sever hemolytic Anemia (Hypochromic microcytic )
2-Chipmunk Rodent face
3-Splnomegaly ;hepatomegaly
4- congestive heart failure
5-Erythroid hyperplasia ➡️crewcut skull X-ray crewcut skull x-ray
Lap finding B thalassemia :
1- Increase microcytic hypochromic
2-Increased reticulocyte count
3-Marked uncjugated
4-Increase serum iron
Treatment of B thalsemia
Supportive treatment for effective erythropoiesis: - Regular blood transfusions:
- minimize anemia - suppress ineffective erythropoiesis
- Iron-chelating agents:
- as Desferrioxamine (DFO) and deferiprone (DFP) - It is Chelating agents acts by binding free iron in the bloodstream and enhancing its elimination in the urine - reduce excess iron → show a significant decline in serum iron - demonstrated great efficacy for cleaning cardiac iron
- Stem cell or bone marrow transplants:
- are the only cure for thalassaemia,, but they are not done very often because of the significant risks involved
Prognosis:
- If Untreated : die during the 1st or 2nd decade
Glucose 6 phosphate dehygence deficiency
X linked recessive disorder
Decrease synthesis and half life enzyme
Pathogensis OF G6PD
G6PD ⬇️ synthesis NADPH ⬇️synthesis of glutathione GSH neutralizes H2O2 oxidant Hb Heinz bodies
1-damage Membrane RBCmembrane intravacscular hemolysis
2- removed RBCs by splenic Extravascular hemolysis
In second baronet half life of G6PD markedly reduced enzyme highest in young RBCs old RBCs are more susceptible to destruction
Lap finding
1-Noromocytic , normochromic
2-peripheral Blood finding
$-Heinz bodies
$Bite cell
3-Hemoglobinuria
Erythroblastosis RH
Etiology &pathogensis
Rh (-) maternal , fetus Rh(+)
IgG gross placenta to the blood fetus hemolysis
This not to first pregnant
Clinical feature of Rh
Treatment
Presentation
Severely affected fetus hydrops fetalis
Heart failure, generalized edema jaundice
T: Exchange blood
P: D immunoglobulin in last month
Test 1 : ESR Erythrocyte sedimentation Rate
- To detect any inflammation - To monitor the progress of inflammatory disease - to evaluate the response to treatment
Dose not spesfic diagnosis