Anemia Flashcards
What is the most common form of anemia?
normocytic
what is normocytic anemia due to?
- decreased production of RBCs ( ACD, aplastic anemia)
- increased destruction of RBCs (hemolysis, posthemorrhagic anemia)
- Uncompensated increased in plasma volume (pregnancy/fluid overload)
Describe hemolytic anemia:
RBCs are destroyed and removed from the bloodstream before their normal lifespan of 120 days. Bone marrow tries to increase RBC production, but it will not be able to match the losses. Homozygous sick cell is the most common cause of hemolytic normoctyic anemias in children.
How is hemolytic anemia classified?
Either intrinsic or extrinsic.
What are intrinsic examples of hemolytic anemia?
- membrane defects: hereditary spherocytosis
- glycolytic defects: pyruvate kinase deficiency
- Oxidation vunerability: G6PD deficieny
- Hemoglobinpathies (sickle cell syndrome)
What are some examples in extrinsic anemia?
- autoimmune
- HUS, DIC
- Infection
- hypersplenism
- burns
What are the signs and symptoms of a hemolytic anemia?
jaundice, fatigue, RUQ and LUQ pain
What will the labs show for hemolytic anemia?
Noromocytic, normal mcv increased retic normal or decreased haptoglobin increased total bilirubin increased serum LDH
How do you treat hemolytic anemia?
Treatment is based on the cause, may need a transfusion.
Describe aplastic anemia:
A condition of bone marrow failure that arises from suppression of or injury to the hematopoietic stem cell. The bone marrow:
- hypoplastic
- fails to produce mature blood cells
- panctyopenia develops
- no abnormal hematopoietic cells seen in blood or bone marrow
What will the labs show for aplastic anemia?
pancytopenia, normocytic RBCs, normal MCV, decreased retic
What is the cause of aplastic anemia?
- autoimmune
- radiation/chemo
- toxins
- drugs
- pregnancy
What are the signs/symptoms of aplastic anemia?
fatigue, weakness, bacterial and fungal infections (decreased neutrophils), skin bleeding (decreased platelets), pallor, purport, petechiae. THERE WILL BE NO BONE TENDERNESS!!!
What is the treatment for aplastic anemia?
EPO or myeloid growth factors, bone marrow transplant.
Describe Anemia of Chronic Disease:
it can be microcytic (<82) or normocytic (82-98). It should be suspected in a patient with a chronic infection or inflammatory process or malignant condition. It is the most common form of anemia in adults.
What are the signs/symptoms of ACD?
It should be suspected in those with a known chronic condition. Fatigue and weakness.
What will the labs show in ACD?
normocytic or microcytic RBCs, decreased retic, decreased serum iron, decreased TIBC, increased serum ferritin, increased ESR or CRP.
What co-anemia do people with ACD often have?
IDA, you should suspect this if there is also a decreased serum ferritin.
What is the treatment for ACD?
address the underlying condition
What are examples of microcytic anemias?
IDA, ACD, chronic lead exposure, thalassemias
describe IDA and what causes it:
The cause is:
- deficient diet of iron
- decreased absorption
- increased requirement
- blood loss (chronic)
- hemoglobinuria
what are the symptoms of IDA?
easy fatigue, tachycardia, palpation, tachypnea. SEVERE deficiency can lead to mucosal and skin changes– smooth tongue, brittle nails, spooning nails and cheliosis.
What will the labs show in IDA?
decreased serum iron
increased TIBC
decreased serum ferritin
low MCV
What is the treatment for IDA?
oral iron replacement, ferrous sulfate. may cause N/V so it should be taken with food, but it is better to take on an empty stomach. Therapy should be continued to 3-6 months.
describe the presentation of chronic lead exposure:
colicky abdominal pain and constipation
HA and irritability
microcytic anemia and basophilic stippling
severe presentation: coma, convulsions
Chronic exposure: learning disabilities, motor neuropathy.
How do you treat chronic lead exposure?
severe: edatate calcium disodium
less severe: oral chelator
Describe the thalassemias:
They are hereditary disorders characterized by a reduction in the synthesis of glob in chains (alpha or beta). Reduced globin chain synthesis causes reduced hemoglobin synthesis and eventually produces a hypo chromic microcytic anemia.
How is thalassemia classified?
minor: lab features without significant impact
intermedia: occasional RBC transfusion requirement
major: when the disorder is life threatening and the patient is transfusion dependent.
Describe Alpha thalassemia:
it is due to gene deletions. It affects all hemoglobins equally side all contain 2 alpha globins. Usually there are 4 genes responsible for alpha glob ins.
3 alpha globins: hematological normal
2 alpha globins : a form of thalassemia minor
1 alpha globin: Hgb H disease
0= dead
Describe Hgb H disease:
chronic hemolytic anemia
PE reveals pallor and splenomegaly
Do not require transfusion generally, only during hemolytic exacerbations life infections and other stressors.
describe Beta thalassemia:
usually caused by point mutations.
results in premature chain termination causing defects in expression of beta-globin.
1. defect causing absent beta globin = beta0
2. defect causing reduced synthesis = beta +
What is homozygous Beta thalassemia?
It is either beta+/beta+ or beta0/beta0
affected children are normal at birth, but develop severe anemia after 6 months, and transfusion is required. Clinical problems include
- stunted grunt
-bony deformities
-hepatosplenomegaly, jaundice and thrombophilia.
What is beta thalassemia intermedia?
same genotype as major but live longer, and do not require transfusion unless there are sever aplastic problems.
What is normal adult hemoglobin made from?
it is 98% hemoglobin a
What are the hallmark lab features of thalassemias?
small and pale RBCs
anemia
normal to increased RBC count
retic can can be increased.
The diagnosis of alpha thalasemia is…
a diagnosis of exclusion
How will Hgb H disease present on lab work?
need to order an Hgb electrophoresis which will reveal the disorder. Peripheral smear will be abnormal: hypochromia, microcytosis, target cells, and poikilocytosis.
How does hematocrit change in regards to the different beta thalassemias?
as beta thalassemia gets more severe (minor–> intermedia–> major), hematocrit falls.
In beta-thalassemia major, what will be the major Hgb present?
Hgb F.
Do you treat the minor thalassemias?
no, only identify
How do you treat Hgb H disease?
folic acid supplements
What is the treatment for severe thalassemias?
regular transfusion schedule and iron chelation.
What is the only available cure for beta-thalassemia major?
allogenic stem cell transplantation.
