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142 Cards in this Set
- Front
- Back
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the rupture or destruction of blood cells releasing Hgb into surrounding environment causing anemia
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Hemolytic anemia
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In hemolytic anemia, the actual anemia occurs when...
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the demand of the body exceeds the capacity of the b.m. to produce RBCs
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red cell membrane defects
enzyme defects hemoglobinapathies Thalassemias |
Intrinsic hemolytic disorders
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immune hemolysis
infection chemicals splenic sequestration |
Extrinsic hemolytic disorders
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by IgG or IgM anitbodies
Activate complement to lyse RbCs |
Intravascular Hemolysis- Immune hemolysis
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phagocytosis of RBCs by fixed phagocytes, spleen, or liver
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Extravascular Hemolysis- Immune hemolysis
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pt. prod. antibodies to foreign RBC antigens introduced by transfusions, pregnancy, or transplants
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Alloimmune- Immune hemolytic anemia
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antibodies produced are directed against the patients own RBCs develops
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Autoimmune- IHA
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Intravascular IHA lab findings
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Hemoglobinemia
hemeoglobinuria decreased to absent haptoglobin increased serum bilirubin increased LD reticulocytes |
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Extravascular IHA lab findings
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spherocytes
elevated serum bilirubin increased urobilinogen decreased haptoglobin DAT positive |
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drug alters the RBC antigens and body doesnt recognize own
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Methyldopa induced
(Drug induced IHA) |
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penicillin and cephalosporins
Drugs absorbed by RBC and elict and antibody response |
Drug adsorption (Hapten)
(Drug induced IHA) |
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pt. responds to certain drugs by forming antibodies to them
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Immune Complex
(Drug induced IHA) |
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intrinsic HA is usually
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hereditary
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extrinsic HA is usually
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almost always acquire
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intrinsic defects
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defects in membrane, enzyme defects, hemoglobinopathies
paroxysmal nocturnal hemoglobinuria |
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extrinsic defects
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IHA
infections chemicals, toxins thermal injury Splenic sequestration |
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due to spectrin or actin deficiency
net loss of cell membrane results in spherocytes |
Skeletal protein abnormalities of RBCs
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leads to formation of acanthrocytes
due to excess free plasma cholesterol on outside of RBC membrane spleen tries to take take out cholesterol and causes misformed shape |
Lipid composition abn. of RBCs
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caused by hereditary spherocytosis, IHA, burns, chemical injury to RBCs
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Spherocytes
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caused by abetalipoproteinemia
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Acanthocytes
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caused by PK def., uremia
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Echinocytes
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caused by microangiopathic anemia
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schistocytes
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seen with osmotically frail spherical RBCs
most common hereditary hemolytic anemia of European descent membrane defect results in decreased surface to volume ratio cells are less deformable |
Hereditary Spherocytosis
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molecular defects involve RBC membrane skeleton
common in Africa |
Common Hereditay eliptocytosis
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part of Herediatry Elliptocytosis
characterized by microsperocytes, micropoikilocytosis, fragments, and few elliptocytes always decreased MCV and elevated MCHC |
Hereditary Pyropoikilocytosis
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deficiency in cell membrane
weakens under stress from circulation cells become distorted and lose shape results in elliptocytes and poikilocytes |
Common Herediatry Elliptocytosis and
Hereditary Pyropoikilocytosis |
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common and asymptomatic in Asian and Cape Cod/ S. Africa
has rigid, spoon shaped ovalocytes resistant to malaria MCV- N to slight increase N- MCH and MCHC |
Southeast Asian Ovalocytosis
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alterations in the permeability of RBC to cation
macrocytosis and stomatocytes |
Membrane Cation permeability disorder
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Seen in Membrane Cation permeability disorder
water follows Na ion into cell, causes swelling |
Stomatocytosis
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seen in Membrane Cation permeability disorder
due to increased eflux of K ion causing cell to dehydrate/shrink target cells are present with small spiculated RBCs RBCs have high Hgb conc in one part of cell |
Xerocytosis
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deficient of dysfunction of metabolic enzymes
inherited RBC prone to early destruction because decr. integrity of cell membrane HMP and EM pathways |
Enzymatic Def. HA
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seen with anemai, reticulocytosis, increased bilirubin. jaundice, poikilocytosis, and decrease in Haptoglobin
diagnosis and 340nm spectropho. |
Enzymatic Def. HA
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maintains conc. of reduced GSH
GSH protects RBC from oxidant damage levels maintained by conv. NADP to NADPH by G6PD |
HM Shunt
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energy of RBC prod by glycolysis
glucose catabolized by lactic acid to prod ATP maintain membrane deformability and normal shape |
EM pathway
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Enzymatic Def. HA lab findings
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autosomal recessive
NC/NC anemia reticulocytosis incr. bilirubin neonatal jaundice DAT positive |
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most common RBC enzyme disorder
X linked 350 variants |
G6PD def
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impaired GSH levels results in accumulation of cellular oxidant
Hgb oxidized to methemoglobin that precipitates to form Heinz bodies spleen removes H.b > schistocytes (bite cells) |
G6PD def
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only seen directly after hemolytic episode:
polychromasia spherocytes microcytic/hypochromic fragments of RBC bite cells reticulocytosis leukocytosis incr. LD lncr. indirect bilirubin decr. haptoglobin |
G6PD def
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most common deficiency of EM pathway
2nd most common RBC enzyme def. |
PK Def.
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catalyzes the conversion of PEP to pyruvate with the conversion of ADP to ATP
deficiency leads to less energy prod. ATP less ATP results in alterations to the RBC membrane, leading to K loss and dehydration (echinocytes) |
PK def
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seen with mild to mod. anemia
splenomegaly jaundice common with gallstones NC/NC anemia reticulocytosis echinocytes, acanthocytes, target cells HJ bodies incr- indirect Hgb and LD osmatic fagility is normal increased Autohemolysis at 48 hours |
PK def
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diagnostic testing
fluorescence persists for 45-60 mins (elevated from normal) |
PK def
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seen with increased RBC destruction
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incr serum unconj. bilirubin
incr- LDH incr- free Hgb incr- methemalbumin decr- HCT, Hgb, RBCs incr- urine urpbilinogen |
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seen in increased RBC prod
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reticulocyte count
incr- MCV, WBCs, PLTs hyperplasia in b.m. |
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seen with most hemolysis
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incr. bilirubin
decr- haptoglobin incr- RPI >2.5 Hgb- 12-13 variable MCV with matching MCH incr- MCHC, > 36, 50% of the time |
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acquired HA
prod of abn RBCs, WBCs, and PLTs makes RBCs more susceptible to complement mediated intravascular lysis usually in young adults always with hemosideriuria |
Paroxysmal Nocturnal Hemoglobinuria
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anemia that results in decr. oxygen carrying capacity of blood
exhibited as hypoxia |
Hypoproliferative anemia
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PLT life span
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7-10 days
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granulocyte life span
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6-12 hours
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RBC life span
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120 days
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most common and most sever bone marrow failure syndrome
due to depletion of b.m. cells and replacement with adipose tissue periph. blood- pancytopenia, but 1st appears as normocytis with reticulocytes b.m.- cells large and uniform, all large and touching together > 50 yo |
Aplastic Anemia
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unknown cause
40-70% of cases |
Idiopathic Aplastic Anemia
(acquired) |
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failure of b.m. due to effects of b.m. microenvironment
-radiation, drugs, abn. immune mech., infections |
Secondary AA (acquired)
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congenital hypoplastic or aplastic anemia with progressive pancytopenia ( not seen until 5-10 years of age)
childhood onset markedly incr. HgbF, HgbA decr. treat with b.m. transplant |
Fanconi Anemia
Primary AA (inherited) |
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rare, x linked
seen with upper body reticulated pigmentosa, dystrophic nails, and leukoplakia 50% develop pancytopenia and hypoplastic b.m. |
Dyskeratosis congenita
Primar AA (inherited) |
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seen with stunted growth, photosensitivity, and telangietic facial erythema
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Blooms Syndrome- Ashkenazi Jews
Primary AA (inherited) |
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hypochromic/ microcytic anemai due to loss of iron in urine
can have thrombocytic complications neutropenia in 3/5 pts thrombocytopenia in 2/3 of pts. causing pancytopenia |
Paroxysmal Nocturnal Hemoglobinuria
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tests for Paroxysmal Nocturnal Hemoglobinuria
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Sucrose hemolysis test
Acidified Serum test (ham test)- definitive diagnosis |
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present with pallor, fatigue, and weakness
normal spleen pancytopenia with N/N anemia no NRBC HGB <7 decreased retics. petichiae, bleeding gums, nose or eyes form thrmobocytopenia neutopenia causes bact. infs. lack of immature cells hypocellular b.m. |
AA
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AA treatment
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blood products (transfusion)
immunosupressive therapy and growth factors bone marrow transplant |
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rare
affecting only erythroid precursors otherwise N b.n. may be hereditary or acquired transient in children can be traced to drugs or infections assoc. with thymoma suggesting an immunologic etiology |
Pure Red Cell Anemia- AA
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rare congenital
erythrocyte aplasia in very young children anemia in 1st year of life but as late as 6 yo no leukopenia or thrmobocytopenia chromosomal abn. |
Diamond- Blackfan syndrome
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familial refractory anemai
ineffective erythropoiesis secondary hemosiderosis abn. of b.m. cells with giantism, multinuclearity, and karyorrhexis |
Congenital Dyserythropoietic Anemia
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normal b.m. cells replaced with abn. cells instead of loss
metastaic tumors military tuberculosis granulomas |
Myelophthisic Anemia
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hematologic disorders that terminate in AL
periphreal blood- pancytopenia, bicytopenia, or isolated cytopenia with decr. retics common macrocytic anemia normocellular or hypercellular b.m. with qualitative abn. of one or more cell lines |
Myelodysplastic Syndromes
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HgbA
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2 alpha
2 beta |
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HgbA2
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2 alpha
2 delta |
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HgbF
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2 alpha
2 gamma |
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1st fetal Hgb chain produced, gestational
slowly decreases at 6 weeks and is gone by 12 weeks (while alpha chain is increasing) |
Zeta chain
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primary Hgb chains seen from 12 weeks to at birth
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alpha and gamma chains
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decrease of ___ chains and increase of ____ chains at birth
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gamma
beta |
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primary Hgb chains, postnatal and through adult life
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alpha and beta
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has increased O2 affinity
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fetal Hgb
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decreased prod. of globin chains
structurally normal effects alpha or beta |
Thalassemias
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result in microcytic anemia
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Thalassemias
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structural abn. in either alpha, beta, or delta chains
impair function due to point mutations |
Hemoglobinopathies
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Hemoglobinopathies-
with increased O2 affinity erythrocytosis |
Hgb Chesapeake
Hgb JCapetown |
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Hemoglobinopathies-
with decr. O2 affinity cyanosis and anemia |
Hgb Seattle
Hgb Vancouver Hgb Mobile |
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Methemoglobinemia
contains ferric rather that ferrous iron cant bind O2 cyanosis |
HgbM Saskatoon and HgbM Kankakee
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unstable hgb
causes heinz bodies |
Hgb Gun Hill
Hgb Leiden Hgb Koln |
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abn- solubility
effect- Hgb precipitation clinical- Hemolytic anemia |
HgbS and C
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having different allelic genes at 1 locus
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heterogenous
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caused by decrease in prod. of alpha globin chains
due to deletion or mutation of one or more alpha globin chian genes at chromosome 16 incr. Beta chains |
Alpha Thalassemia
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have 3 functional genes taht code for prod. of alpha globin chain
-a/aa no symptoms test with DNA |
Silent carrier/ Alpha Thal
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have 2 functional genes that code for the prod. of alpha globins
-a/-a or --/aa as cis or as trans |
Alpha Thal minor
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tends to be in Asian descent
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cis- Alpha Thal
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tends to be in Africans
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trans Alpha Thal
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Alpha thal with mild microcytic anemia
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-a/-a
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Alpha thal with more sever microcytic anemia
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--/aa
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mild microcytic, mypochromic anemia
MCv < 80 |
Alpha Thal
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has gamma tetramers
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Hgb Barts
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has beta tetramers
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Hgb H
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3 deleted genes
--/-a Alpha thal-1/ Alpha thal-2 chronic hemolytic anemia |
Hgb H
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homozygous alpha thal 1
4 delted genes --/-- lethal |
Hgb Bart's
hydrops fetalis |
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B0 B0
no B chains tranfusion dependent |
Beta Thal major
Cooleys anemia |
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B+ B or B+ B0
significant anemai may require transfusion |
Beta Thal intermedia
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B+ B
microcytosis, erythrocytosis, mild if any anemia |
Beta That trait
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Beta Thal-
Ineffective erythropoiesis causes |
marrow hyperplasia
frontal bossing, maxillary hyperplasia |
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Beta Thal-
Hemolytic anemia causes |
splenomegaly
high output CHF |
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Beta Thal-
Decreased O2 carrying capacity causes |
microcytosis
hypochromia |
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microcytosis and erythrocytosis
target cells 2 alpha chain deletion |
Alpha Thal
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microcytosis and erythrocytosis
target cells need Hgb A2 level to distinguish B+ B |
B Thal
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aka leptocytes
seen in Thalesseias, almost any hypochromic anemias, liver disease, Hemoglobinopathies |
Target cells
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seen in large numbers in Hgb SS, Beta and Delta Beta Thal trait
seen in most cells in Hgb EE, CC, Thal major, and HgbH |
Target cells
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B6(A3) Glu:Lys
microcytic, 40% total Hgb homozygous, mild HA |
HgbC
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False Neg in Sickle Solubility Test
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sever anemia, Hgb < &
low % of HgbS, newborns, transfusion |
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alkaline
all hgb migrates to anode (+) |
Cellulose acetate eletrophoresis
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Acid
migraton based soley on charge fro Hgb S, C, E |
Citrate Agar Electro.
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on gel with a pH gradient
hgb travels to isoelectri point and stops better separation |
IEF
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B121 (Gh4) Glu:Gln (D Los Angeles/Punjab)
in British and Punjab normal CBC migrates with S on alkaline gel |
Hgb D
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fast mover on alkaline
Heavy Hb F- neonates Alpha Thal |
Hgb Barts
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MCV > 100
incr- MCH N- MCHC |
Macrocytic Anemia
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anemia due to ineffective erythropoiesis resulting from disrupted DNA synthesis
effects all marrow elements |
Megaloblastic Anemia
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macrocytic with ovalocytes
hyperseg neutrophils pancytopenia decr. retics incr. LDH B12/ folate decr megaloblastic changes in b.m. |
Megaloblastic Anemia
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mild to mod. anemia
MCV up to 110-130 MCH 33-38 |
periphreal blood in Megaloblastic Anemia
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affects bone marrow and epithelial cells
nuclear maturation lags behind cytoplasm maturation |
Megaloblastic Anemia
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causes subactue degeneration of nervous system
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B12 def.
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Vit B12 aka
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Cobalamin
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B12 adsorption req._____
secreted by parietal cells protects B12 during transport down GI tract |
Intrinsic factor
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nutritional def. or decr. supply of B12 (Kwashiorkor)
pernicious anemia Chrons Castles IF def |
Macrocytic Anemia- B12 def
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causes for b12 def
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gastric failure
ileal failure competing organisms |
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requires presence of appropriate receptors in ileum to facilitate
need functinal and adequate transalcobalamins to move to storage sites and to cells |
B12 adsorption
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periphreal blood- Megaloblastic anemia
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macro-ovalocytes
incr RDW poikilocytosis RBC inclusions (HJ, basophilic stippling) megaloblastic RBCs decr. retics |
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bone marrow- Megaloblastic B.M.
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giant bands and metamyelocytes
hemosiderin granules in macros |
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neurologic pattern for B12 def
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peripheral neuritis: numbness and tingling of fingers/ toes
impaired sense of position and vibration (Rombergs sign) spasticity, hyperactive deep reflexes, positive toes signs weakness psychiatric symptoms |
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vit B12 def. from atrophy of stomach lining resulting in no or little intrinsci factor
in European and african ancestry |
Pernicious Anemia
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gray hair
light blue, gray eyes blood group A between 50-60 yo |
PA
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weakness, sore smooth tongue, numbness and tingling in extremities
lips, gums, and tongue appear bloodless loss of appetite, weight loss, diarrhea, pain inchest lemon colored skin (pallor and icteric) high pulse |
PA
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megaloblastic feature of B12 def.
decr. RBC survival decr. haptoglobin incr. methemalbumin |
PA
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Lab tests for PA
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Gastric analysis
Scholling Test- definitive measure Methylmalonic acid anf homocysteine |
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aka pteroylglutamic acid
present in food uptake req. B12 as cofactor absorbed in jejunum |
Folate (Folic acid)
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caused by
poor diet incr. requirement drug therapy malabsorption |
Folate Def
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Folate def. clinical process
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serum folate decr
hyperseg neutrophils urinary excretion of FIGLU anemia |
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abn. absorption due to celiac disease or sprue
incr. utilizaton caused by pregnancy or AL treat with antimetabolites |
Macrocytic Anemia- Folate Def.
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seen in alcoholic pt. with poor diet
infant malnutrition complication of sickle cell of thalessemia drugs tropical sprue |
Macrocytic Anemia- Folate Def.
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peripheral blood- MA
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giant bands and metas
hyperseg neutrophils- classic indicator |
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bone marrow- MA
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giant metamyelocytes always found in MA
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MA without megablastosis
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Mypothyroidism
Sever liver disease alcoholism hemolysis AA with macrocytes normal WBC and PLT |
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on Beta chain
substitution on chrom 11, position 6 subs. of valine for glutamic acid |
Hgb S
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on Beta chain
substitution on chrom 11, position 6 subs. of lysine for glutamic acid |
Hgb C
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on Beta chain
substitution on chrom 11, position 26 subs. of lysine for glutamic acid |
Hgb E
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