Anemia Flashcards
anemia
reduction in Hb concentration or decrease in number or RBC/mm
-too low to deliver O2 to meet cellular demands
what type of anemia can be seen in the newborn period?
blood loss, hemolytic dz of the new born
what can cause the decreased production of RBC?
Hb synthesis
Fe deficiency
Thalassemia
Anemia of chronic dz
DNA synths
Aplastic anemia
myeloproliferative leukemia
Bone marrow infiltration
carcinoma
lymphoma
pure red cell aplasia
what can cause increased production of RBC?
blood loss
intrinsic hemolysis
hereditary spherocytosis
Elliptocytosis
Sickle cell
unstable hb
G6PD defi
extrinsic hemolysis
warm/cold antibody
TTP-HUS
mechanical cardiac valve
clostridial infxn
hypersplenism
how to you classify anemia?
decreased production of RBC (reticyocyte count low)
increasd destruction of RBC (hemolysis)
Red cell loss (reticuloctye count high)
early infancy anemia causes
pure red cell aplasia, physiologic anemia
6 mnth to 12 years anemia
nutritional anemias, acute inflammation (illness) bone marrow infiltration
signs and sx of anemia
** few physiologic distrubance < 7-8
decreased O2 transport- fatigue, dyspnea, syncope
decreased blood volume - pallor, postrual hyptension
increased cardiac OP_ congestive heart failure
what is the most usueful lab for anemai?
reticulocyte count for the cause of anemia
what are you looking at during a blood smear?
- normocytic, microcytic, macrocytic
- normochromic, hypechromic, hyop
+/- spherocytes, schistocytes,
maybe see polychromasia
direct coombs test
prenatal testing of pregnant women and in testing blood prior to a blood transfusion.
microcytic hypochromic anemia
Iron def
lead poisoning
thalassemia (minor-beta/alpa)
beta-thalassemia
Iron def anemia
the most common during childhood. Usually seen between 6-24 months, but not uncommon during adolescence. More common during periods of rapid growth and higher potential for inadequate dietary iron
if pt is chronically taking ASA/NSAids
maybe contributing to iron def
CF of iron def
o With moderate (Hgb 6-8 g/dL) pallor, fatigue, irritability, tachycardia, cardiomegaly, systolic murmurs (LSB), pica, decreased appetite, decreased exercise tolerance
-Pica is the hallmark of iron deficiency
-Severe deficiency (Hct < 25%) :
brittle nails, cheilosis, smooth tongue, formation of esophageal webs (Plummer-Vinson syndrome
Plummer-Vinson Syndrome
“The Plumber Vincent DIGS a hole for the IRON pipie”
D= dysphagia from esophageal webs
I=Iron def anemoa
G=Glossitis
S= squamous cell carcinoma
IRON=tx
think severe deficiency!
lab findings in fe def anemai
Decreased Hgb, MCV, ferritin
- Increased TIBC
- Hypochromic, microcytic RBC. Anisocytosis and poikilocytosis
anisocytosis
Anisocytosis is a medical term meaning that a patient’s red blood cells are of unequal size. Th
poikilocytosis
Poikilocytes are abnormally shaped red blood cells
management of Iron def
o Mild to moderate anemia
Therapeutic trial of oral iron therapy
• Ferrous sulfate, 325 mg tid – given in slow escalating doses. Absorption enhanced by vitamin C
• Repeat hemoglobin after 1 month (should normalize)
• Continue iron replacement for 3-6 months after achieving a normal hemoglobin
• If patients are intolerant to oral iron, have GI disease, or continuing blood loss parenteral iron. Sodium ferric gluconate is less likely than iron dextran to cause anaphylaxis
Dietary modifications limiting milk
microcytic anemia lead poisoning
lead poisoning
often concomitant w/ iron def.
lead poisoning
inhibition of sythetic enzymes necessary for production of hemoblobin
lab findiing on lead poisoning
Low MCV, basophilic stippling on smear
sideroblastic anemia?
the bone marrow produces ringed sideroblasts rather than healthy red blood cells (erythrocytes).
thalassemia
- Characterized by varying degrees of ineffective hematopoiesis and increased hemolysis, resulting in decreased or absent globin synthesis
- The most prominent feature is microcytosis out of proportion to the degree of anemia
how is thalassemia dx
electrophoresis
CF of thalassemia?
- Hemoglobin usually between 3-6 g/dL
- Serum iron and ferritin are usually normal (or elevated
what population has thalassemia
with African, Mediterranean (beta), Middle Eastern, Chinese (alpha) or Southeast Asian (alpha) ancestry
what causes Betat T
point
what causes alpha T
gene
beta thatlassemia major
a transfusion-dependent condition
a-thalassemia
- Severity of clinical syndrome depends upon number of genes expressed
what happens w/ a single gene deletion in a thalaseemia
silent carrier
two gene deletion for alpha Two gene deletion in A. thalassemia
Occurs in 1.5% African Americans
Results in hypochromic, microcytic anemia (mild)
Normal Hgb electrophoresis
what happens when there is a 3 gene deletion?
o Hemoglobin H disease (on electrophoresis)
o One α-globin chain
o Moderately severe chronic hemolytic anemia
o Variably symptomatic
o Need folic acid supplements and should avoid iron supplements and oxidative drugs (e.g., dapsone, primaquine, quinidine, sulfonamides, nitrofurantoin)
4 gene deletion or A. thal.
o Bart’s or Hydrops fetalis (and thus, still birth)
o Incompatible with life :severe anemia, tissue hypoxia, heart failure, hepatosplenomegaly, edema
B-Thalassemia (mino and major)
results from an abnormal beta gene in one or both of the genes, occasionally entire gene is deleted
BT minor
only one gene
-lifelong anemia
HgbA2 and HgbF
no tx needed
Cooley’s anemia
both gnese needed for beta globin production are affected
-ineffective erthyropoieses
CF of Cooley’s eanemai
o Severe anemia, organomegaly, growth failure (failure to thrive is prominent)
Can get frontal bossing, maxillary hypertrophy with prominent cheekbones, overbite
o Basophilic stippling. Hemoglobin electrophoresis shows Hgb F and Hgb A2
o Problems begin at 4-6 months, when the switch from fetal hemoglobin (Hgb F) to adult hemoglobin (Hgb A) occurs.
tx of Cooley’s
life long transfusions of packed RBC
what is the main cause of death from Cooley’s?
cadiace disease!
what is the goal after blodd transfusions? (in cooley’s
(Goal 14.5g/dL)
• Allow improved growth and development
• Reduce hepatosplenomegaly
• Reduce bone deformities
Macrocytic anemia
larger than avg RBC
*also acute hemorrhage and hemolysis
Macrocytosis
MCV >100
- down syndrome
- normal newborn
- folic acid def
- vit b12 def
how is macrocystosis subclassifed?
based on presence of absence of megaloblastosis
- megaloblastic: vitamin B12 deficiency, folate deficiency, drugs, metabolic disorders
- anemias w/ MCH > 125 almost always megaloblastic ex for those associated w. myelodysplastic syndrome
what is non-megaloblastic
bone marrow failure syndromes (Diamond-Blackfan, Fanconi), chronic liver disease, hypothyroidism
what lab work ups should you use?
- peripherla blood smear
- B12 level
- folate level
- bone marrow bx
vit b12 deficiency
pernicious anemia-leads to atrophic gastritis and increased risk of gastric carcinoma
what food has vit b12 in it?
meat, fish, cheese, eggs
where does vit b 12 absorption occur
terminal ileum and storage in the liver
what can cause pernicious anemia?
malabsorption, strict vegan diet, gastric surgery, pancreatic insufficiency, Crohn’s disease
what are CF of pernicious anemai
- Glossitis, pale icterus, diarrhea, weight loss, vitiligo, possible neuro manifestations (stocking-glove paresthesias, loss of position, fine touch, and vibratory sensation, clumsiness, dementia, and ataxia
what other lab results are associated w/ pernicious anemia?
hypersegmented neutrophils,
serum LDH and indirect bilirubine
decreased reticuloctyes count
Folate deficiency causes?
poor dietary intake- alos defective absorption, prego, hemolyytic anemia, alcohol abuse, and consumption of of folic acid antagonists (i.e., phenytoin, TMP/SMX, sulfasalazine)
CF of folate def
sore tongue (glossiltis) and vague GI sx
lab finding in folate def
macro-ovalocytes and hypersegmented polymorphonuclear cells
dx: <150 mg/nL
what are other findings in folate def?
Howell-Jolly bodys (nulear DNA remnants)
tx of folate def
1 mg folate
Diamond-Blackfan anemia
- Congenital pure red cell aplasia
- May have congenital anomalies short stature, web neck, cleft lip, shield chest, triphalangeal thumb
- Macrocytosis and reticulocytopenia
- Treat with oral corticosteroids or transfusion if necessary (maybe even bone marrow transplant)
Fanconi Anemia
- autosomal recessive, results in pancytopenia
- what is the mean agor of onset? 8yrs
what are some clinical features of fanconia anemia?
- Hyperpigmentation and café au lait spots, microcephaly, microphthalmia, short statue, horseshoe or absent kidney, absent thumbs
how do you tx fanconi anemia
transfusions, even stem cell transplant
normocytic anemia
associated w/ chronic inflammatory dz
- caused by organ failure or impaired marrow fxn resulting from systemic dz
- upregulation of hepcidin in response to inflammatory mediators
*** LOW reticulocyte response
types of normocytic anemia
Anemia of inflammation (results from chronic inflammatory dz- such as JRA)
-congenital pure red cell aplasia
- Transient erythroblastopenia of childhood
- acquired pure red cell aplasia
- gradual onset of pallor
- self-limited w/in 1-2 mnths
hemolytic anemia
-episodic or continuous RBC destruction
what is hallmark of the hemolytic
elevated reticuloctye count in the present of a falling or stable hematocrit
peripheral smear of hemolytic anemai?
immature red cells, nucleated red cells, or morphologic changes
Intrinsic hemolytic anemia
- intrinsic membrane defect
- RBC enzyme defects
- Hemoglobinopathes
acquired hemolytic anemaia?
fragmentation hemolysis
-immune-medicated hemolytic anemia
Hereditary hemolytic anemia
- Intrinsic membrane defects
- RBC Enzyme Defects
- Hemoglobinopathes
intrinsic membrane defects
o Conditions caused by abnormalities of the RBC cytoskeleton which produces accelerated RBC destruction in the spleen
o Elasticity is what makes red cells able to deform reversibly
o If they cannot reshape to biconcaved discs, then there is an destruction of erythrocytes through the spleen
Hereditary spherocytosis
- Autosomal dominant
- Elevated MCHC
- Spherocytes on peripheral smear-Hemolysis occurs in the extravascular compartment and the spleen (possible splenectomy if severe)
- May have indirect hyperbilirubinemia
how do you dx herediarty spherocytosis?
osmotic fragility test
hereditary elliptocytosis
benign dominantly inherited disorder
elliptocytes on peripheral smear
-significant hemolysis 10% pt
RBC ezyme defects
G6PD def
common worldwide
gene is X-lined recessive disorder
oxidative drugs (ASA, dapsone, primaquine, quindine, sulfonamides, macrobid)
what will you see on a blood smear of someone who has G6PD def?
Heinz body (denatured hb), bite cells
what is the type A variant of G6PD
10% of AA males
- Hemolysis can occure w. antimalarial agents
- can be protective agains malaria
Hemoglobinopathies
Sickle Cell Dz
HgB S gene-carried by 8% of AA population
Homozygous SS, SC, SB thal are clinically significant syndromes
• Lifelong anemia
• Acute and chronic tissue damage related to sickling
• Increased susceptibility to encapsulated organisms
• SA is sickle cell trait
how do you dx hemogobinopathies
Hb electrophoresis
HbSC dz?
- HEP SC
- Hgb 9.0 – 12.0 gm/dL
- MCV 60 – 80 fL (normal)
- Retics 3 – 5%
- Smear: microcytosis, hypochromia, target cells
HbSS dz?
most common and severe form
- HEP: SF
- Hgb 6.5 – 8.5 gm/dL
- MCV 80 – 100 fL (normal)
- Retics 5 – 15%
- Lifespan shorted to 10-20 days (normal is 120)
- Smear : sickled cells, polychromasia, Howell-Jolly bodies, poikilocytes, target cells
• Sickling is increased by red cell dehydration, acidosis, and hypoxemia
what are howell-Jolly bodies are indicative of?
hyposplenism
what can make sickling worse?
red cell dehydration, acidosis, hypoxemia
Sickle Cell Dz
hypchromic, microcytic
what are acute clinical manifestations of SCD
-Acute vasoocculusive episodes (pain crises, stroke, acute chest syndrome)
what is the first sign of SCD?
dactylitis “sausage fingers”
Actue anemic episodes
splenic sequestration, aplastic crises (human parvovirus)
Infection and SCD
increased susceptibility secondary to fxn asplenia
- PCN prophylaxis
- Pneumovax
what is asplenia
absence of normal spleen function and is associated with some serious infection risks.
Chronic clinical manifestations
CNS, CVD respiratory, hepatobiliary, ocular, skeletal, nutritional, GU, growth and development
what other issues may pple w/ SCD present w/
unconjugated hyperbilirubinemia, elevations of LDH and AST
Transfusion Indications in sickle cell dz
- pre-anesthesia prep
- stroke prophylaxis
- sever acute chest syndrome
- aplastic crisis w/ severe anemia
- splenic sequestration crisis
- elevated TCD velocity
Hydroxyurea Therapy in scd
-improves hematological values
-increases fetal Hb which decreases sickling
-results in fewer:
painful events, episodes of acute chest syndrome, erythrocyte transfusion,
hematopoietic stem cell transplantation and SCD
the only potential cure for SCD
-limited by lack of donor availabiltiy and toxicides or procedure
acquired hemolytic anemias
- HIGH reticulocyte response
- fragmentation Hemolysis
- immune-mediated hemolytic anemia
Types of Fragmentation hemolysis
TTP HUS giant hemangioma artifical heart valves Sepsis DIC
Immune-mediated Hemolytic Anemia
- hemolytic dz of the newborn
- autoimmune hemolytic anemia
Aplastic anemia
an acquired failure or hematopoietic stem cells that result in pancytopenia
-injury or abnormal expression of the pluripotent hematopoietic stem cell
-
what infxn may cause an aplastic crisis?
parvovirus B19
Clinical features of aplastic anemia?
fatigue, pallor, thrombocytopenia (bleeding, easy bruising)
Weakness, petechia,
***Hepatosplenomegaly, lymphadenopathy, and bone tenderness suggest an alternative diagnosis
dx features of aplastic anemia
**Pancytopenia
- microcytosis
- bone marrow replaced by fat (hypocellular bone marrow)
what can cause aplastic anemia?
- idiopathic in developed countries
- may be induced by certain druges or infx (hepatitis and mononucleosis)
how to tx severe cases
HSCT w. sib
mild cases of aplastic anemia?
supportively w/ transfusion of red cells and platelets
