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130 Cards in this Set

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Site of iron absorption
Duodenum
Cells which regulate iron absorption
Mucosal (of duodenum)

if iron deficient iron passed to body, if not it may be sloughed back into gut
3 stages of iron deficiency disease
1 iron dificiency without anemia (limited stores)

2 Iron limited hematopoiesis (production of microcytic cells as body runs out of iron)

3 Iron deficiency anemia
Hypocrhomic anemia: differential diagnosis (7)
1 Iron deficiency--95%

2 beta thalassemia trait

3 Sideroblastic anemia

4 Lead poisoning

5 Anemia of chronic disease

6 Alpha thalassemia

7 Hypercuprinemia
Lab Characteristics of beta thalassemia trait (3)
1 hypochromic anemia

2 iron studies normal

3 basophilic stippling
Lead poisoning lab characteristics (2)
1 hypochromic

2 coarse basophilic stippling
Peripheral smear w/ iron deficiency (5)
1 hypochromic

2 anisocytosis

3 poikiliocytosis

4 +/- target cells

5 NO basophilic stippling
Serum iron vs TIBC in

Iron deficiency
Iron is low

TIBC is high
Serum iron vs TIBC in

anemia of chronic disease
iron is low

TIBC is low
iron deficiency is 95% certain if Fe/TIBC is?
< 10%
if Fe/TIBC 10-30%, then
iron deficiency must be distinguished from anemia of chronic disease
If ferritin <10, then
iron deficiency
If ferritin 10-30, then
possible iron deficiency
Clinical features of iron deficiency (4)
1 Pallor, conjunctival pallor (not specific--signs of anemia)

2 koilonychias (spoon nails)

3 angular chelosis

4 glossitis
Treatment of iron deficiency
Ferrous sulfate 300 mg t.i.d.

--60 mg of elemental iron per pill

--NO enteric coated iron
Duration of iron deficiency treatment
until hematocrit is normal, then 3-6 months to replete marrow stores
Treatment of iron deficiency with malabsorptive patients
IM injection (not IV) of iron
Conditions with decreased soluble transferrin receptor
conditions of erythroid hypoplasia, such as aplastic anemia and red cell aplasia

anemia of chronic disease
conditions with increased soluble transferrin receptor
conditions of erythroid hyperplasia, such as chronic hemolytic anemia

in iron deficiency
soluble transferrin receptor use for differential of iron deficiency and anemia of chronic disease
increased in iron deficiency

decreased in anemia of chronic disease

--useful when iron, TIBC, and ferritin are borderline
soluble transferrin receptor/log ferritin

in iron deficiency vs anemia
of chronic disease

(#?)
iron deficiency: >4

anemia of chronic disease: <1
screening test for hemochromatosis
transferrin saturation

NOTE: high saturation will be seen in aplastic anemia b/c of inability to use iron
Mutations associated with hemochromatosis
C282Y, H63D, S65C

NOTE: C282Y +/+ confirms dx
definition of megaloblastic
increase in early erythroblasts in the bone marrow
most common causes of macrocytic anemia
folate deficiency and B-12 deficiency

NOTE: these two are also megaloblastic b/c they cause slow DNA production and therefore slow maturation
Folate role in DNA synthesis
methyl donor in thymidine synthesis
Where is folate absorbed
the jejunum
folate body stores vs B12 body stores duration
folate lasts few months, B12 for 3 years

Thus, if diet interupted folate defiency first
Drug which causes folate malabsorption
dilantin (aka phenytoin), anti seizure drug
Conditions of increased folate need
pregnancy and hemolytic anemia
Tests specific for folate deficiency (2)
red cell folate

serum folate (not as good, since one meal may raise serum, but not red cell folate)
Blood smear and marrow in folate deficiency
smear: macrocytic with hypersegmented neutrophils

megaloblastic marrow
Therapy for folic acid deficiency
Folic acid 1 to 5 mg/day
Danger of folic acid replacement therapy w/o B12 evaluation
Blood counts may correct, but neurologic problems may progress
Role of B-12
via role in methionine synthesis, converts methyl-THF to THF
steps of B12 absorption (3)
1 stomach: binds to R factors, protects it from acid degradation.

2 Duodenum: pancreatic enzymes free B12 from R binders, binds to intrinsic factor (from parietal cells)

3 B12-IF absorbed by terminal ileum
Conditions associated with pernicious anemia (5)
1 premature gray hair

2 Vitiligo

3 Thyroid disease

4 Hypoparathyroidism

5 Gastric atrophy w/ achlorhydria
Causes of B12 deficiency
Diet (1)
vegan diet, no eggs, cheese, or meat
Causes of B12 deficiency
Gastric (5)
1 pernicious anemia

2 Decreased IF

3 Atrophic gastritis

4 gastrectomy

5 Zollinger-Ellison (inc acid, no binding to IF)
Causes of B12 deficiency:
Intestinal (4)
1 regional enteritis
2 celiac disease
3 pancreatic disease (no enzymes to liberate from R binders)
4 blind loop

--also fish tapeworm, rare
Clinical features of B12 deficiency (5)
1 pallor w/ lemon yellow skin (due to hemolysis in marrow)

2 Glossitis (beefy tongue, megaloblastic tongue)

3 paresthesia of hands and feet, position and vibration sense loss

4 combined system disease (demylenization of dorsal and lateral columns)

5 other neuro: somnolence, altered taste and smell, scotomata, dementia
Dx of b12 deficiency based on serum B12
<100 diagnostic
100-200 suspicias
>200 normal
test to establish diagnosis and pathophysiology of B12 deficiency
Schilling test

Give radioactive B12 by mouth

2 hrs later, give a flushing dose of B12 I.M. (1 mg)

measure urine radioactivity vs feces. If in urine, then it was absorbed.

Repeat with oral intrinsic factor
Therapy for B12 deficiency
1000 micrograms/day x 2 weeks, then weekly until hematocrit is normal. Brisk reticulocytosis occurs within a week.
METHYLMALONIC ACID TEST, why?
Since B12 is required to convert methylmalonate to succinate, some physicians advocate testing for urine methylmalonic acid as a diagnostic test for B12 deficiency
MACROCYTIC ANEMIA – the full differential diagnosis(8)
1 Folate and B12 deficiency are 95% or more of cases

2 Chemotherapy with antimetabolites

3 Alcohol – even in the absence of folate deficiency

4 Liver disease

5 Hypothyroidism

6 Myelodysplasia (usually a normocytic anemia, can be macrocytic)

7 INCREASED RETICULOCYTES – Each 1% elevation of retics raises the MCV 1 point.

8 Myeloma and dysproteinemias (artefactual). Cells clump together and the machine counts two cells as one.
Differential: anemias of bone marrow failure(6)
1 APLASTIC ANEMIA

2 PURE RED CELL APLASIA

3 MYELOFIBROSIS

4 MYELODYSPLASIA

5 MYELOPHTHISIC ANEMIA

6 ANEMIA OF CHRONIC DISEASE
Dx of aplastic anemia
bone marrow biopsy showing aplasia
tx of aplastic anemia (2)
Immune therapy with anti-lymphocyte serum, cyclosporine, and cortiocosteroids

bone marrow transplant
Pure red cell aplasia dx
Low hematocrit

LOW (or zero) reticulocytes

NORMAL wbc and platelets

Marrow: NO red development, everything else normal
Two possible causes and one association of pure red cell anemia
Cases may be drug related (dilantin), or may occur post infection (with parvovirus) or in association with thymoma.
Tx of pure red cell aplasia
corticosteroids, immunosuppressive therapy

blood transfusions, iron management.

very survivable
myelofibrosis (aka agnogenic myloid metaplasia) dx
splenomegaly (can be MASSIVE)

teardrop cells on blood smear give a hint. white count usually normal--can be elevated, resembling CML

biopsy showing fibrosis is diagnostic (aspirate often "dry tap")
types of myelodysplasia with high rate of leukemia development
RAEB--refractory anemia with excess blasts

RAEB-T--refractory anemia with excess blasts in transformation
myelodysplasia dx
anemia

Pathologic sideroblasts containing more than five iron granules per cell

low reticulocyte count
MYELODYSPLASIA
group of clonal disorders, present with anemia, half of the patients eventually die of leukemia, others have marrow failure without leukemia at the time of death
MYELOPHTHISIC ANEMIA
Anemia due to bone marrow replacement usually by a tumor
Diagnosis?
RBC smear: Normal
Marrow: Hypoplasia
WBC: Decreased
Differential: Normal or decreased polys
Platelets: Decreased
Aplastic Anemia
RBC smear Normal
Marrow No RBCs
WBC Normal
Differential Normal
Platelets Normal
Pure Red Cell Aplasia
RBC smear Teardrops
Marrow Fibrosis
WBC Variable
Differential Normal or increased Myelocytes and other immature WBCs
Platelets Normal or Low
Myelofibrosis
RBC smear Normal
Marrow Ineffective RBC Production
WBC Variable, normal or low
Differential Normal, Increased blasts when advanced
Platelets Normal: Decreased in advanced stages
Myelodysplasia
RBC smear Nucleated RBCs
Marrow Infiltration by tumor or other process
WBC Variable
Differential Variable: normal or may resemble myelofibrosis (inc myelocytes and other immat. Wbc's)
Platelets Decreased
Myelophtisis
RBC smear Normal
Marrow Normal
WBC Normal
Differential Normal
Platelets Normal
Anemia of Chronic Disease
drug that delays progression of myelodysplasia to acute leukemia
Vidaza
Cytogenetics more likely to be abnormal in which two myelodysplasias
RAEB (Refractory anemia with excess blasts) and RAEB-T (Refractory anemia with excess blasts in transformation)
subtypes of myelodysplastic syndrome
Refractory anemia RA
Refractory anemia with ringed sideroblasts RARS
Refractory anemia with excess blasts RAEB
Refractory anemia with excess blasts in transformation RAEB-T
Chronic myelomonocyctic leukemia CMML
most prominent feature of myelodysplastic syndromes ("often")
Anemia
cell lineages involved in myelodysplastic syndromes
Red cells, granulocytes and platelets are involved
Therapies that do not work for myelodysplasia
13 cis retinoic acid

Corticosteroids

Androgens

Low dose cytosine arabinoside
interpretation of reticulocyte count vis a vis hemolytic anemia
increased in hemolytic anemia, does not PROVE hemolysis

Bleeding causes increased reticulocyte count

NOTE: Concommittant problem limiting red cell production (i.e. parvo virus injury of marrow) can lower rets
How do you know if anemia is hemolytic? (5)
1 Reticulocyte count is elevated

2 Bilirubin is up and unconjugated

3 LDH (lactic dyhydrogenase) will be elevated

4. HAPTOGLOBIN: down (if intravascular, not spleenic hemolysis)

5. Decreased chromium 51 survival (rarely measured)
Classes of hemolysis (where is the defect?) (4)
1 Outside the red cell (IgG "warm", IgM "cold", hypersplenism, other)

2 Membrane defects (spherocytosis)

3 Enzyme defects (90% G6Pd--oxidant sensitive; pyruvate kinase 10%)

4 Abnormal hemoglobin
Dx of immune hemolytic anemia
1 Positive Coombs test (direct version)

2 lab determines which type of antibody or complement (IgG warm, IgM cold)
Treatment of warm antibody hemolysis (3)
1 corticosteroids. if fails..

2 splenectomy. if fails..

3 immunosuppression
Treatment of cold antibody hemolysis (3)
1 keep patient warm, blood products warm

2 Plasmapheresis (b/c IgM intravascular)

3 Cytotoxic therapy

--NO splenectomy or steroids
conditions associated with IgG hemolysis (3)
1 systemic lupus

2 chronic lymph leuk

3 lymphoma
conditions assoc with IgM hemolysis (3)
1 mycoplasma infection

2 infectious mono

3 Waldenstrom's macroglobunemia
3 mechs of drug caused hemolytic anemia
1.Antibody against a drug which binds to the membrane when present in very high concentration – occurs with high dose pencillin.

2.Neoantigen formation, the antibody is against a drug/membrane complex This is the most common mechanism of drug related hemolytic anemia

3.Aldomet mechanism. Autoimmune hemolytic anemia like idiopathic process occurs. No need for drug to be present.
chinese:spherocytosis
球形红细胞症

qiúxíng hóngxìbāo zhèng

"spherical RedCell disease"

spherocytosis
كرية حمراء كروية
Red globule spherical

spherocytosis
球形红细胞症

qiúxíng hóngxìbāo zhèng
qiúxíng hóngxìbāo zhèng

"spherical RedCell disease"

spherocytosis
spanish: spherocytosis
esfirocitosis
arabic spherocytosis
كرية حمراء كروية

Red globule spherical
WARM ANTIBODY hemolytic anemia
1 molecule bound to cell?
2 where macrophage binding?
3 which organ injured?
4 Injury extra or intra vasc?
5 Rx?
1 IgG
2 Macros bind in cords of Billroth in spleen
3 Splenic destruction
4 extravascular
5 Corticosteroids, splenectomy
COLD ANTIBODY hemolytic anemia
1 molecule bound to cell?
2 where macrophage binding?
3 which organ injured?
4 Injury extra or intra vasc?
5 Rx?
1 Complement--fixed by transient IgM
2 Liver
3 Hepatic injury
4 Intravascular
5 Keep warm, plasmapheresis, cytoxic chemotherapy
Hereditary spherocytosis most common defect
spectrin (membrane protein)
hereditary spherocytosis Rx
splenectomy
Diagnosis of Sickle cell
Hemoglobin electrophoresis – mostly S, some F, some A2, no A

NOTE: If A is present it is either sickle thalassemia (S>A) or sickle trait (A>S)
血红蛋白

xuèhóng dànbái
xuèhóng dànbái

bloodred eggwhite (protein)

hemoglobin
screening test for sickle cell diesease or trait
sodium metabisulfite
Blood smear of thalassemia (5)
1 Microcytic anemia
2 hypochromia
3 anisocytosis
4 target cells
5 basophilic stippling

NOTE: basophilic stippling does NOT occur in Fe deficiency
Thal trait vs Fe deficiency
Iron normal, iron binding normal, Hgb A2 elevated (3.5% vs 2.5%), basophilic stippling
葡萄糖六磷酸鹽脫氫酶缺乏症

pútaotáng liù línsuānyán tuō qīng méi quēfá-zhèng
pútaotáng liù línsuānyán tuō qīng méi quēfá-zhèng

grape-sugar 6 phosphorous-acid-salt break hydrogen enzyme(ferment) lack-disease

Glucose-6-phosphate dehydrogenase deficiency
ttp, who cares?
Without treatment 80% die, with treatment, plasmapheresis,

80-90% are cured.
اِسْتِخْراج بلازما
plasmapheresis
血浆取出法
xuèjiāng qǔchū fǎ
xuèjiāng qǔchū fǎ

blood-syrup takeout method

plasmapheresis
Mechanism of PAROXYSMAL COLD HEMOGLOBINURIA
in cold, IgG binds to rbc's. when warm, cells lyse.
Coomb's negative for IgG, + for complement.
Cross reactivity w/ syphilis?
erythrocytosis differential
pseudoerythrocytosis--low plasma volume

secondary erythrocytosis--"reactive" to erythropoietin--could be physiologic or abnormal

polycythemia vera
abnormal causes of erythropoietin elevation (6)
1 renal disease
2 CNS disease
3 liver disease
4 cushings
5 adrenal adenoma
6 uterine fibroids
diagnostic criteria of polycythemia vera
Red cell mass (volume) Males >36 ml/kg; Females > 32 ml/kg

Oxygen saturation >92%

bone marrow with trilineage hyperplasia
3 steps of evaluation of elevated hematocrit
1 if hematocrit >60, then not pseudoerythrocytosis. If < 60, start with red cell volume and plasma volume.

2 2ndary erythrocytosis vs. P vera. Test for common 2ndary causes (hypoxia, CO) Perform
A Complete Blood Counts
B Arterial blood gases with CO level
C Bone marrow aspiration and biopsy

3 If no diagnosis, then look for exotic causes:
Perform
A Renal ultrasound
B Pelvic and abdomen CT
C Oxygen dissociation (abnormal hemoglobin)

NOTE: phlebotomy can treat P Vera and 2ndary so not always necessary to definitively diagnose
Treatment of pseudoerythrocytosis
Volume repletion
Treatment of secondary erythrocytosis
treat underlying disorder (hypoxia, etc)
phlebotomize to hematocrit under 48
Tx of polycythemia vera
Phlebotomy <70
P32 (radioactive phosphorus) for older
A1 and A2 diagnostic criteria of polycythemia vera
A1: Raised red cell mass (volume) more than 25% above mean normal predicted value, or hematocrit > 60 in males or > 56 in females

A2: Absence of cause of secondary erythrocytosis
PURE RED CELL APLASIA
An autoimmune disease in which an antibody to early erythrocytes is present.
ANEMIA OF BONE MARROW FAILURE
common lab features
1 normocytic anemias
2 low reticulocyte count
3 crucial diagnostic test in this situation is the bone marrow aspirate/biopsy
APLASTIC ANEMIA
initially thought to represent toxic, irreversible damage to bone marrow stem cells

low hematocrit, platelet, granulocytes

immunosupression often works, so autoimmune
MYELOFIBROSIS
A clonal myeloproliferative disorder in which a stem cell exists which grows better outside the marrow, in the spleen
ANEMIA OF CHRONIC DISEASE
1 A non-specific term used to describe those cases in which a chronic condition is associated with an anemia for which no specific cause can be found.

2 Inflammation could mute response to erythropoietin
low vs high reticulocyte count in anemic patient. MCV significance
If high hemolytic or blood loss

If low, anemia of bone marrow failure--will increase MCV
Tests for iron deficiency
1 Peripheral smear--hypochromic w/ anisocytosis and poikilocytosis, NO basophilic stippling

2 Serum iron/serum iron binding capacity. Low for iron deficiency

3 Serum ferritin, proportional to body stores.

4 Bone marrow definitive, but $ and ivasive
most common cause of iron deficiency
Bleeding is most common cause of this anemia
what is the biochemical problem of sickle cell anemia
Amino acid substitution as 6 position of beta chain, glutamine to valine
Most common anemia(s) with following morphology: macrocytic
Morphology associated with: Folate deficiency
Most common anemia(s) with following morphology: Normocytic (w/ low reticulocytes)
Morphology associated with: Pure red cell aplasia & Myelodysplasia
Most common anemia(s) with following morphology: Hypochromic
Morphology associated with: Iron deficiency
Most common anemia(s) with following morphology: microcytosis
Morphology associated with: Thalassemia
Most common anemia(s) with following morphology: Dimorphic micro and macrocytes
Morphology associated with: sideroblastic anemia
Most common anemia(s) with following morphology: Schistocytes
Morphology associated with: Microangiopathic hemolytic anemia
Most common anemia(s) with following morphology: Target cells
Morphology associated with: Iron deficiency, splenectomy, thalassemia
Most common anemia(s) with following morphology: Teardrop cells
Morphology associated with: Myelofibrosis
Morphology associated with: Folate deficiency
Most common anemia(s) with following morphology: macrocytic
Morphology associated with: Pure red cell aplasia & Myelodysplasia
Most common anemia(s) with following morphology: Normocytic (w/ low reticulocytes)
Morphology associated with: Iron deficiency
Most common anemia(s) with following morphology: Hypochromic
Morphology associated with: Thalassemia
Most common anemia(s) with following morphology: microcytosis
Morphology associated with: sideroblastic anemia
Most common anemia(s) with following morphology: Dimorphic micro and macrocytes
Morphology associated with: Microangiopathic hemolytic anemia
Most common anemia(s) with following morphology: Schistocytes
Morphology associated with: Iron deficiency, splenectomy, thalassemia
Most common anemia(s) with following morphology: Target cells
Morphology associated with: Myelofibrosis
Most common anemia(s) with following morphology: Teardrop cells