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RUTICULOCYTE COUNT :
ANEMIA :
decrease in hemoglobin +- decrease in RBC and haematocrit
c/p :
- 0-symptoms of anemia : due to hyper dynamic circulation : palp - sob -
fatigue ,Pallor of skin , nails and MM.
- 1-Signs of anemia :growth failure
- 2- glossitis and angular stomatitis
- 3-koilonychia
- 4-brittle thin hair and may be associated with plummer-vinson syndrome
- 5-pica (case about someone eating unusual stuff)
laboratory findings:
Rayyan.T
- CBC :Microcytic hypochromic anemia (↓ MCV, ↓ MCHC , ↑RDW -> mentzer
>13)
Leukocyte count is usually normal.
- - The platelet count is often raised
- -Retculocytopenia if not treated
- iron studies :
- 1- ↓ in BM hemosidren (first to ↓)
- 2- ↓ serum ferritin (< 30 ng/mL)
3- normal or ↓ serum iron (due he is taking from storage)
4- ↑ total iron-binding capacity (TIBC) (N: 300 μg/dL)
- 5-↓ % saturation of iron binding protein (transferrin )
Hx of pallor : ODP , localized or generalized - associated symptoms -who notice it
-ROS -PSH - FH of anemias
Case 1 : infant with low HB and low MCV and MCHC and high RDW
DX: microcytic hypochromic anemia Due to iron deficiency due to high RDW
(indcates some are small and affected and some is normal *characteristic*)
-Very important : always asses for 3 in PBF always lock to lymphocyte —> if RBC is
smaller than it —> decrease mcv or bigger than lymphocyte —> increase MCV.
Then lock at pallor inside if more than 1/3 —> decrees MCHC , and notice RDW is it high
or normal
- normal MCV rule = 70 + age —> bellow that is abnormal.
- Normal hb rule = 11 + age/10
- ex age is 4y -> cutoff value is 11.4 if he is below it its abnormal.
- MENTZER RATIO = MCV / RBC *NO 0* —> IF ABOVE 13 —> iron deficiency , if
Bellow -> B-thalassemia
Management : مهم
-iron supplements 4-6mg/kg/day for 3 months (rbc span)
-response to ttt:
1- irritability goes (1-2d)
2-reticulocyte count increase (2-3d)
3- hb level increase (4-30d)
4- ferritin increase (1-3m)*last responder*
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2-Megaloblastic anemias
appearance in BM of abnormal large red cell precursors called megaloblasts.
Pathogenesis:
- Folate or vitamin B12 deficiency → Impaired DNA synthesis → Delayed nuclear maturation
→ Causes a block in cell division → leading to large, nucleated hematopoietic cells called
Megaloblasts
So,
- RBCs have megaloblastic maturation in BM→ form macro-ovalocytes in PBF
- WBC have Giant metamyelocytes in BM → Hypersegmented Neutrophils
(>5 lobes) in PBF.
Causes of Vit B12 deficiency:
1- Dietary deficiency:
- Rare (because B12 is stored in liver & sufficient for years),but seen in strict
vegetarians
2- ↓ absorption:
a- ↓ intrinsic factor (IF) : gastrectomy or pernicious anemia
b- terminal iliuem:, Crohn's disease of ileum , celiac , bowel
surgery
hints :
-goat milk —> folate deficiency -> no cns
symptoms
- vegetarian - bowel problem(celiac and
IBD)—> b12 deficiency —> cns symptoms
3-Fancconi anemia :
Def : AR inherited bone marrow failure syndromes (IBMFs)
c/p :
1-Skin :café au lait spots, petechiae and bruises with later onset of pallor due
BM failure
2-microcephaly
3- absent / abnormal thumb
4-short stature and failure of growth
Lab : pancytopenia with high MCV.
Confirm dx :
-chromosomal breakage at FANCA gene at chromosome F-16 *most important*
-gene mutation search
Complication : inc Risk of leukemia so you have to do bone marrow biopsy
every year. **
dx : aplastic anemia(ospe) :
—> bone marrow replaced by fatt —> 100 - age = cellularity of bm
Causes : viral hepatitis - facconi - drugs and toxin: benzene / chloramphenicol -
radiation - idiopathic.
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ttt: 1-blood products ( not given to normalize to normal )
2-abx
3-immunosuppresion
4-bone marrow transplant
case 3 : 7y boy with short stature and absent thumb and low hb and wbc —>
facconi anemia
4-Physiological anemia :
Physiologic phenomena at beginning of life due:
1- life span of rbc in infant is 90 days
2- low EPO level due to it was very high in utero because of hypoxia —> over oxygenation
of tissue
*Hb cutoff of physiologic anemia in:
-term : not less than 9
-pre term = not less than 8*