Anemia Flashcards
_____ means a low mean corpuscular volume (MCV) <80
Microcytic anemia
Microcytic anemia causes
These are most commonly a result of iron deficiency, anemia of chronic disease, thalassemia, sideroblastosis, and lead poisoning
_____ is characterized by an elevated MCV >100.
Macrocytic anemia
MCC of Macrocytic anemia
This is most commonly
from vitamin B12 or folic acid deficiency but can also result from the toxic effects of
alcohol, liver disease, or chemotherapeutic agents such as methotrexate or medications
such as zidovudine (AZT) or phenytoin
Packed RBCs are used to maintain a hematocrit______
> 25–30%.
Normocytic anemia, Reticulocyte Count <3%
(bad bone marrow response) ddx
Early stages iron deficiency anemia
• Early stages anemia of chronic disease
• Aplastic anemia
• Chronic renal failure
Iron absorption is tightly regulated. A man requires about ______ per day and a woman about ____mg per day on average.
1 mg
2–3
As the hematocrit approaches 30%, symptoms _______
of fatigue and poor exercise tolerance may develop.
As the hematocrit lowers to 25%, ______
tachycardia, palpitations, dyspnea on exertion, and pallor develop
A_____may develop in any patient with
moderately severe anemia
systolic ejection murmur (“flow” murmur)
Symptoms specific to iron deficiency are rare and cannot be relied upon to determine the
diagnosis. These include ______
brittle nails, spoon shaped nails, glossitis, and pica.
A______ is the most characteristic finding of iron deficiency anemia
low serum ferritin <10 ng/mL
Low ferritin has good specificity ___but poor sensitivity ____
(>99%)
(60%);
The serum iron is____ and the total iron binding capacity is ____. The RDW is elevated.
low
high
A defect in the ability to make use of iron sequestered in stores within the reticuloendothelial system. It can be either microcytic or normocytic
Anemia of Chronic Disease
____ a regulator of iron metabolism, plays an important role in anemia of
chronic disease
Hepcidin,
In states where hepcidin level is abnormally high (e.g., inflammation), serum
iron falls due to ____
iron trapping within macrophages and liver cells and decreased gut iron absorption.
Hepcidin inhibits iron transport by binding to the iron export channel _____
located on the surface of gut enterocytes and the plasma membrane of macrophages
ferroportin
In genetic diseases where hepcidin level is abnormally low, what are the CX?
iron overload may occur (hemochromatosis) due to unwarranted ferroportin facilitated iron influx.
Dx of Anemia of chronic DSE
The serum ferritin level is normal or elevated. The serum iron level and total iron binding capacity (TIBC) are both low. The reticulocyte count is low
Anemia of chronic DSE TX
Iron supplementation and erythropoietin will not help, except in ______
renal disease and anemia caused by chemotherapy or radiation therapy
A microcytic anemia caused by a disorder in the synthesis of hemoglobin characterized
by trapped iron in the mitochondria of nucleated RBCs
Sideroblastic Anemia
Sideroblastic Anemia
The hereditary form is from either a
defect in_____ or ____
aminolevulinic acid synthase or an abnormality in vitamin B6 metabolism
Sideroblastic Anemia
Acquired forms are from drugs such as ______. Lead poisoning can cause
sideroblastic anemia as well.
chloramphenicol, isoniazid, or alcohol
Sideroblastic anemia may progress to _____in a small percentage of patients
acute myelogenous leukemia
Dx of sideroblastic anemia
The serum ferritin level is elevated.
Transferrin saturation is very high, and therefore the TIBC is very low. The serum iron level is high
Sideroblastic anemia
The most specific test is a _____
Prussian Blue stain of RBCs in the marrow that will reveal the ringed sideroblasts
Sideroblastic anemia is the only microcytic anemia in which _____
serum iron is elevated.
Sideroblastic anemia Tx
Some patients, especially those with
INH-associated sideroblastic anemia, will respond to______
pyridoxine therapy 2-4 mg per day
The hereditary underproduction of either the alpha or beta globin chains of the
hemoglobin molecule resulting in a hypochromic, microcytic anemia
Thalassemia
Alpha thalassemia is more common in____
Beta thalassemia is more common in ___
Asian populations.
Mediterranean populations
In alpha thalassemia, 1 gene deleted yields a normal patient. What are the CBC, Hb and MCV levels?
The CBC is normal, the
hemoglobin level is normal, and the MCV is normal.
Alpha thalassemia
Individuals with 2 genes deleted have a mild anemia with hematocrits ranging from _____ with a strikingly____
30–40%
low MCV.
Alpha thalassemia
Those with 3 genes deleted have more profound anemia with hematocrits _____ as
well as the very low ____. Four-gene-deleted alpha thalassemia patients die in utero
secondary to gamma chain tetrads called ______
22–32%
MCV
hemoglobin Barts.
These patients with beta thalassemia major,
also known as Cooley anemia, become severely symptomatic starting at 6 months because?
the age when the body would normally switch from fetal hemoglobin to adult hemoglobin
Cx of beta thalassemia major,
They are later symptomatic from hemochromatosis, cirrhosis, and CHF from chronic anemia and transfusion dependence.
Clues to the diagnosis of thalassemia trait is a ________
mild anemia with a profound
microcytosis
_____differentiates which type of thalassemia is present.
Hemoglobin electrophoresis
Hb electrophoresis
In beta thalassemia, there is an increased level of_______
hemoglobin F and hemoglobin A2.
Those with alpha thalassemia will have normal amounts of hemoglobins F and A2. Tetrads of beta chains are called ___
hemoglobin H.
Hemoglobin H is present in _____
alpha thalassemia with 3 of 4 genes
deleted.
____ are present in all forms of thalassemia trait and thalassemia major.
Target cells
Thalassemia traits of both the alpha and beta types do not require specific treatment.
Beta thalassemia major patients require____
blood transfusions once or twice a month
Thalassemia
The chronic transfusions lead to iron overload, which requires treatment with _______
deferasirox. Oral deferasirox is the standard of care.
The most common cause of B12 deficiency is ______ which is a disorder resulting in decreased intrinsic factor production due to autoimmune destruction of parietal cells.
pernicious anemia,
Various forms of malabsorption such as ____, _____, _____ can block absorption of vitamin B12.
sprue, regional enteritis, and blind loop syndrome
DX of Anemia from B12 def
The WBCs have ______
The red cells are characterized by____
hypersegmented neutrophils with a mean lobe count >4.
macro-ovalocytes
B12 and folate deficiency produce ____. The hematologic pattern of vitamin B12 deficiency
is indistinguishable from folate deficiency.
oval macrocytes
The most specific test is a____.
_____confirm the etiology as pernicious anemia.
low B12 level
Antibodies to intrinsic factor and parietal cells
An elevated ____ occurs with B12
deficiency and is useful if the B12 level is equivocal
methylmalonic acid level
Tx of Vit B12 def
Parenteral route is recommended for patients with_____. IV dosing is not recommended because that would result in most of
the vitamin being lost in the urine.
neurologic manifestations
of B12 deficiency
Response of vitamin B12 deficiency anemia to treatment is usually rapid, with reticulocytosis
occurring within_____
2–5 days and hematocrit normalizing within weeks
Vit B12 def
Treatment with cobalamin
effectively halts progression of the deficiency process but __
might not fully reverse more
advanced neurologic effects.
Patients who have vitamin B12 deficiency with associated megaloblastic anemia might experience severe_____and fluid overload early in treatment due to increased erythropoiesis, cellular uptake of potassium, and increased blood volume
hypokalemia
____can correct the hematologic abnormalities of B12 deficiency, but not
the neurologic abnormalities
Folic acid replacement
Folic acid def
Occasionally, increased requirements from pregnancy, skin loss in diseases like ___
eczema, or increased loss from dialysis and certain anticonvulsants such as phenytoin may occur
Hemolytic anemia
Hereditary Membrane problems
heriditary spherocytosis, hereditary elliptocytosis
Hemolytic anemia
Hereditary Metabolism: problems
G6PD deficiency, pyruvate
kinase deficiency
Hemolytic anemia
Hereditary Hemoglobin:
genetic abnormalities (Hb S, Hb C, unstable)
Hemolytic anemia, acquired
- Autoimmune: ______
- Alloimmune: ______
- Drug-associated
warm antibody type, cold antibody type
hemolytic transfusion reactions, hemolytic disease of the newborn, allografts (especially stem cell transplantation)
_____may occur if the intravascular hemolysis is particularly rapid.
Fever, chills, chest pain, tachycardia, and
backache
Patients with hemolytic anemias generally have a normal MCV, but the MCV may
be slightly elevated because _____
reticulocytes are somewhat larger than older cells.
In HA
There should not be bilirubin in the urine because ____
indirect bilirubin is bound to
albumin and should not filter through the glomerulus
Patients with chronic hemolytic anemia need to be maintained on _____ as there is an increase in cell turnover.
chronic folic acid therapy,
A hereditary form of chronic hemolysis ranging from asymptomatic to severe,
overwhelming crisis. It is characterized by irreversibly sickled cells and recurrent painful crises
Sickle Cell Disease
____is due to a substitution of a valine for glutamic acid as the sixth amino acid of the beta globin chain
Hemoglobin S
A sickle cell acute painful crisis can be precipitated by______
hypoxia, dehydration, acidosis, infection, and fever. However, the crisis may occur without the presence of these factors
Sickle cell crisis is usually not associated with an _____
increase in hemolysis or drop in hematocrit.
Causes of hemolysis in pts with sickle cell crisis
When increased hemolysis occurs, another etiology such as concomitant glucose
6 phosphate dehydrogenase deficiency (G6PD) or acute splenic sequestration in a child should be considered.
A sudden drop in hematocrit may also be caused by Parvovirus B19 infection or folate deficiency
SSx of sickle cell crisis
Chronic manifestations include renal concentrating defects (isosthenuria),
hematuria, ulcerations of the skin of the legs, bilirubin gallstones, aseptic necrosis
of the femoral head, osteomyelitis, retinopathy, recurrent infections from Pneumococcus or Haemophilus, growth retardation, and splenomegaly followed in adulthood by autosplenectomy
Severe manifestations of sickle cell crisis
acute chest syndrome consisting of severe chest pain, fever, leukocytosis, hypoxia, and infiltrates on the chest
x-ray.
sickle cell crisis
Priapism can occur from infarction of the _____
prostatic plexus of veins
The ____ is the most specific test for SCD
hemoglobin electrophoresis
The______ is a quick screening test used to diagnose evidence of sickle cell trait and cannot distinguish between trait and homozygous disease.
sickle prep (or Sickledex)
An acute sickle cell pain crisis is treated with ______
fluids, analgesics, and oxygen
Sickle cell crisis
_____ is the preferred agent because
it covers Pneumococcus and Haemophilus influenza.
Ceftriaxone
Sickle cell crisis
_____ is used to decrease the
frequency of the vaso-occlusive pain crisis.
Hydroxyurea
Various forms of acquired hemolytic anemias resulting from the production of
IgG, IgM, or activation of complement C3 against the______
red cell membrane
AIHA
The lysis can be either extravascular or intravascular but is far more often
____
extravascular
Sites of extravascular hemolysis
Destruction of the cells most often occurs
through macrophages in the spleen or by Kupfer cells in the liver
AIHA
The most common drugs are the ____
penicillins, cephalosporins, sulfa drugs, quinidine, alphamethyldopa, procainamide, rifampin, and thiazides
____can also lead to autoimmune hemolytic anemia
Ulcerative colitis
______ is an IgM antibody produced against the red cell in association with malignancies such as lymphoma or Waldenstrom macroglobulinemia and infections such as Mycoplasma or mononucleosis.
Cold agglutinin disease
Cold agglutinin destruction occurs predominantly in the ______
liver
Cold agglutinin disease results in _____
cyanosis of the ears, nose, fingers,
and toes.
AIHA Dx
Autoimmune hemolysis gives a
normocytic anemia, reticulocytosis, increased LDH, absent or decreased haptoglobin, and increased indirect bilirubin, as can all forms of hemolysis
The _____ is the specific test that diagnoses autoimmune, cold agglutinin,
and often even drug-induced hemolysis
Coombs test
AIHA
_____are often present on the smear
Spherocytes
More severe autoimmune hemolysis is treated with______
steroids first.
Cold agglutinin disease is primarily managed with ______
avoiding the cold
Most cases of cold agglutinin disease are mild, but in those who have severe disease despite conservative measures,____ can be used
azathioprine, cyclosporine,
or cyclophosphamide can be used. Rituximab is also useful
_____ and ____ don’t work well with cold agglutinin disease because the destruction
occurs in the liver
Steroids and splenectomy
A chronic mild hemolysis with spherocytes, jaundice, and splenomegaly from a
defect in the red cell membrane
Hereditary Spherocytosis
An autosomal dominant disorder where the loss of _____ in the red cell membrane
results in the formation of the red cell as a sphere, rather than a more flexible and
durable biconcave disc
spectrin
