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Hemonc test 1 Anemia
NOVA orlando fall sem
72
Medical
Graduate
11/27/2010

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Term
what are two microcytic anemia?
Definition
Iron deficiency and anemia of chronic disease
Term
what are two normocytic normochronic anemia
Definition
aplastic and chronic disease
Term
two megaloblastic macrocytic anemia
Definition
vit B 12 deficiency
folate deficiency
Term
give 6 condition anemia caused by defective Hgb synthesis (hemoglobiinopathies)
Definition
Sickle cell disease
Sideroblastic
Thalassemia
Porphyria
Lead Poisoning
G6PD
Term
what is iron overload anemia called?
Definition
Hemochromatosis
Term
myeloproliferative disorders --- hint PCV
Definition
polycythemia Vera
Term
Episodic or continuous destruction of RBCs
--- due to intracorpuscular defects causes 4 conditions?
Definition
G6PD deficiency*
sickle cell syndromes*
methemoglobinemia
spherocytosis
Term
Episodic or continuous destruction of RBCs

due to Extracorpuscular causes:
Definition
drug toxicity autoimmune diseases*
TTP* lymphoproliferative diseases
HUS* valve hemolysis
DIC* metastatic adenoCa
vasculitides burns
Term
megakaryoblast would become_______
myeloid stem cells would become _____
Definition
thrombocyte
neutrophil, eosinophil, basophile-mast cell
Term
what are young erythrocytes called?

Destruction of stem cells is called?

Norm maturation depends on vit B12 & folate deficiencies is called?
Definition
reticulocyte

aplastic anemia

Megaloblastic anemia
Term
inability to produce globin chain is what condition?

inability to incorporate stored Fe to make heme --- ________________
Definition
thalassemias

Anemia of chronic disease -
Term
give name

cells with ↓ conc of Hb-->
abnormal cell shape -->

crescent shaped cell-->

dark center & periphery with a clear ring in between (bullseye)-->

fragmented, irregularly contracted -->
Definition
Microcytosis: cell size < 6µm
Macrocytosis: cell size > 8µm
Hypochromia: cells with ↓ conc of Hb
Poikilocytosis: abnormal cell shape
Spherocytosis: spherical, w/o pale centers
Sickle cells: crescent shaped cells
Target cells: dark center & periphery with a clear
ring in between (bullseye)
Schistocytes: fragmented, irregularly contracted
Term
normal Hgb and Hct for Female and male
Definition
Hgb – measure of total amt Hgb in peripheral blood. Reflects # RBCs in blood
norm: males 14-18 g/dl
females 12-16 g/dl

Hct – (AKA packed cell vol) measure of % of the total blood vol that is made up of RBCs
norm: males 42-52%
females 37-47%
Term
normale reticulocyte count
Definition
Reticulocyte count – to determine bone marrow function
norm: adult/child 0.5-2% of RBCs
Term
Reticulocyte index determines What?
Definition
RI – reticulocyte index. Determines if retic count indicates an appropriate erythropoietic response from RBC marrow.
normal RI : 1.0
Term
iron is absorbed where in GI and when is absorption increased or decreased
Definition
Is absorbed primarily in duodenum
Balance is tightly regulated by the body
↑ absorption: anemia
hypoxia
↑ erythropoiesis
↓ iron stores
↓ absorption: inflammation
↑ iron stores
Term
most common causes of microcytic anemia?

what stores the iron in liver, spleen and bone marrow?
Definition
Iron deficiency anemia

Ferritin. Once absorbed, iron is transported to tissues by transferrin.
Term
serum iron measures what ?
Definition
↓ serum iron – measures amt of iron bound to transferrin. Evaluates total body iron.
50 – 150 µg/dL
Term
this are example of what disease

Ex: ABO incompatibility, transfusion rxn, thermal burns, snake bites, malaria, bacterial infections, Mycoplasma pneumonia, drugs
Definition
Auto Immune Hemolytic Anemia


From coating of RBC membrane with antibodies &/or complement.

Mediated by: IgG “warm” antibodies
IgM “cold” antibodies
Term
Auto immune hemolytic Anemia coomb's test--- explain direct
Definition
Direct (antiglobulin test)
To ID hemolysis or a hemolytic transfusion rxn
Direct: aids in evaluating suspected transfusion rxn by detecting antibodies that coat transfused RBCs
Uses pt’s blood
Antibodies can develop:
from drugs (levodopa, methyldopa). Antigens develop on the RBC
with some diseases (autoimmune disease, lymphoma)
idiopathic
+ = agglutination
Term
indirect coomb test detect antibody circulating in the blood or the one that coats transfused RBCs
Definition
Indirect: (Indirect antiglobin test)
Detects circulating antibodies against RBCs
To detect minor serum antibodies (other than ABO/Rh system) to RBCs that will be given in a future blood transfusion
Term
type and screen is done in direct or indirect ...when is it done
Definition
indirect and it is done b4 surgery
Term
with autoimmune or lymphoproliferative disorders what kind of antibody? is it warm or cold
Definition
Warm. IgA antibodies react @ body temp
Term
cold Abs
Immune Hemolytic anemia occurs in what group of pt? what kind of abs?
Definition
elderly

Occurs post infection (EBV, Mycoplasma pneumonia)


IgM antibodies bind @ lower temps, usually in distal circulation & bind complement
Term
hallmark for Aplastic anemia. what are the causes of aplastic anemia?
Definition
Pluripotent stem cells in bone marrow are injured → ***pancytopenia*** (hallmark)
Causes: Cause: inherited (Fanconi’s anemia)
radiation
toxins (benzene, carbon tetrachloride)
drugs (chloramphenicol,dilantin, chemo drugs,
acetazolomide, carbamazapine)
insecticides, fungicides
idiopathic
Term
what happens in graft vs host disease ?
Definition
Occurs with grafting of immunocompetent lymphocytes from bone marrow transplant to an immunocompromised host
These lymphocytes divide spontaneously
only takes a small number
Prevention: irradiate all blood products for these pts
Term
what is the tx for aplastic anemia?
Definition
bone marrow transplant
Term
anemia of chronic disease could happen form what other chronic disease
Definition
From chronic renal failure, malignancies, liver,
endocrine or thyroid failure
Term
Anemia of chronic disease --what happens to kidney?
Definition
CRF: as renal endocrine function declines, erythropoietin production is impaired. As kidneys fail to excrete, hemolysis and bone marrow depression occur.
Term
cause and S&S of anemia of chronic disease
Definition
Cause: ↓ EPO production
S&S: fatigue, pallor
tachypnea, tachycardia
 pericardial effusions
Term
Anemia of CRF would show what kind of RBCs? what is the tx?
Definition
Burr cells. recombinant human erythropoietin
Term
lack of intrinsic factor with gastric atrophy would causes what kind of anemia?
what kind of pt would get it? what kind of cancer has increased risk?
Definition
B12 Deficiency anemia.

 in: strict vegetarians*
elderly
post gastrectomy*
surg resection of distal ileum
Crohn’s disease*
Tropical Sprue
alcoholics

gastric cancer
Term
this anemia is dx by doing schilling test (radioisotopes of B12). S & S are sore tongue and angular stomatitis. symmetric paresthesias of feet and hands
Definition
Pernicious anemia
Term
what is the tx for pernicious anemia?
Definition
Rx:  B12 (cyanocobalamin) 1mg/day in  doses till Hct is norm, then 1mg/mo IM for life
monitor serum K+ levels* (po K+ )
Term
______________ are associated with neural tube defects, abnormalities of heart, urinary tract and limbs in neonates
Definition
dec folate level
Term
what two types causes need for folate needs to increase?
Definition
pregnancy and exfoliative skin disease
Term
what does alcohol do to folate --increase level or dec level in body
Definition
dec -- Usually associated with alcoholism. Alcohol interferes with intestinal absorption of folic acid & ↓ hepatic storage
Term
s & S of folate deficiency anemia
Definition
S&S: memory changes
irritability
personality changes
Term
very important what would see on blood smear which will dx folate deficiency anemia ---- two things
Definition
macro-ovalocytes & hypersegmented PMNs are pathognomonic*
Howell-Jolly bodies* (small remnants of
nuclear material in RBCs)
Folate level <150 ng/ml*
Serum B12 levels are normal
Term
x-linked recessive disorder which affects black males and sephardic jews.
Definition
G6PD
Term
what kind of cells and bodies would you see in G6PD that would confirm the dx?
Definition
G6PD level during the illness
bite cells
Heinz bodies
 retic count
 serum indirect bilirubin
Term
what are some causes of G6PD?
Definition
Fava beans, primaquine, quinidine, sulfonamides ASA, soy products, anti-malarials durgs.
Term
what is HbSS?
and What is HbAs
Definition
HbS is defective gene for sickle cell. HbSS is when all of the HbA (normal gene) is replaced by HbS (Defective one). they are positive for sickle cell disease
HbAs: is when they have both. --Sickle cell trait
Term
causes of sickle cells anemia?
Definition
Cause: fever/infection
cold weather
high altitudes
dehydration
acidosis
hypoxemia
Term
what could be long term sequela?
Definition
splenomegaly/asplenia
renal failure
avascular necrosis of femoral head(osteomyelitis)
strokes
priapism
retinopathies→ blindness
Term
what would you notice on lab work to Dx sickle cell?
Definition
Anisocytosis/ poikilocytosis

howell-jolly bodies
sickle cells/target cells
 serum bilirubin, urobilinogen,urobilin
hemoglobinuria
 WBCs
thrombocytopenia/DIC can develop
Term
what is the rx of choice for sickle cell?
Definition
stem cell transplant.

other tx: analgesics (acetaminophen)
supportive – fluids, O2
hydroxyurea
clotrimazole
possible exchange transfusions


Hydroxyurea – switches fetal Hgb gene back on
HbF does NOT sickle
Term
this Anemia is Genetically transmitted.
Decreased or absent synthesis of the α- or β-globin chains→ abnorm cells with ↓ survival rates→ anemia.

BQ-----**Prominent feature is microcytosis out of proportion to the degree of anemia.**
Definition
Thalassemia
Term
Deficiencies of globin chain synthesis produce inadequate hemogoblin which then produce what kind of RBCs
Definition
small (Microcytic)
Term
why does spleen further causes worsening anemia in thalassemia trait?
Definition
When there is a deficiency in alpha-globin synthesis, unused beta-globin chains accumulate in the RBC injuring the cell membrane.
These abnormal RBC’s are then removed from the circulation prematurely by the spleen – further worsening the anemia.
Term
what ethnicity is affected ?
Definition
Asian Mediterranean, and African
Term
what other disease could thalassemia would be confused by due to dec MCV?
Definition
Iron deficiency. however, RDW would be decreased in iron def.

thalassemia has normal RDW.
also order iron level in serum to r/o iron deficiency.
Term
what shape cells for thalassemia?
Definition
Tear shaped, oval cells, microcytic
Term
this anemia is Caused by defects in the incorporation of iron into the heme molecule?
it is a rare anemia. and refractory to Fe therapy
Definition
Sideroblastic anemia--- Sidero means iron
Term
how would you Dx sideroblastic anemia?
Definition
Bone marrow bx.

demonstrate abnormalities in erythropoiesis and the accumulation of iron in erythroblast (ring sideroblasts)
Serum iron is often elevated
Term
what color ring would you see around sideroblasts?
Definition
Blue granules denote ringed sideroblasts (iron)
Term
what causes Sideroblastic anemia?
Definition
inherited (rare)
typically it is an acquired disorder caused by exposure to certain drugs & toxins
ethanol, lead, isoniazid, chloramphienicol
secondary to malignancy of chronic inflammatory diseases
Term
pt comes in complaining of Nerve damage, severe photosensitivity, liver disease and anemia. what could it be
Definition
Poryphyria
Term
vesiculo/bullous eruptions
plaquelike scar formation
hyperpigmentation
excess hair to face
pt with all this would have what kind of porphyria?
Definition
PCT: porphyria cutanea tarda
Term
begins in
childhood
itching, burning, erythema
angioneurotic, edemalike swelling of
exposed skin areas

pt with all this would have what kind of porphyria
Definition
EPP: erythropoietic porphyria
Term
name three enzyme that are inhibited by lead in production of heme?
Definition
PBG synthase
Heme Sunthase
Coproporphyrinogen oxidase
Term
what else does lead do to RBCs?
Definition
Lead appears to injure the RBC membrane, possibly by inhibiting ATPase → impaired cation exchange
Synthesis of α & ß globin chains appear to be defective
Term
Dx of Lead poisoning is done when what is seen on smear?
Definition
normocytic, hypochromic anemia
----RBC stippling

----ringed sideroblasts on peripheral
smear (with severe anemia)

 urine, blood lead levels
Term
lead poisoning S&S?
Definition
Children: speech & language deficits
learning problems
Adults: GI distress (constipation, vomiting)*
severe, diffuse abd pain with episodes of
paralytic ileus (acute intoxication)*
**** linear blue-black line in gingiva****
motor neuropathies
CNS damage
fatigue (anemia)
Term
normal RBC count in male? Female?
Definition
RBC count: males: 4.3 – 5.7 x10³/µL
female: 3.8 – 5.1 x10³/µL
Term
Normal Hgb in male and female
Definition
Hgb: males 14 - 18g/dL
females 12 – 16g/dL
Term
normal Hct in male and female
Definition
Hct: males 37 – 47%
females 35 – 45%
Term
MCV MCH and MCHC normal values
Definition
MCV: 82 - 98µm
MCH: 30- 40pg/cell
MCHC: 31 – 37d/dL
Term
Reticulocyte count:???
Definition
0.5 – 2.5% of RBCs
Term
normal WBC?
Definition
WBC count: 5000 – 10,000/mm³
Term
second most numbered WBC?
Definition
Lymphocytes - 20-40%
Term
platelets normal value?
Definition
150,000 – 350,000/µ/L
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