Heme Flashcards
term for -
RBC’s that stick together like a stack of coins due to increased plasma proteins (immunoglobulins)
Roluleaux formation
Roluleaux formation is seen in what disease
Multiple Myeloma
or increase fibrin disorder like infection or inflammatory autoimmune disease
Term for:
Clumping of RBC’s due to IgM coating the surface leading to RBC destruction by macrophages
Cold agglutination autoimmune hemolytic anemia
Cold agglutination autoimmune hemolytic anemia is seen in what condition ?
Mononucleosis
Key terms for this diagnosis- Spherocytes with negative coomb’s test
+ osmotic fragility test
Herditary spherocytosis
Key terms for this diagnosis- Spherocytes with positive coomb’s test
Warm autoimmune hemolytic anemia
Impaired DNA synthesis with normal RNA synthesis will increase cytoplasmic proteins leading to what cell line?
Macrocytosis- with hypersegmented neutrophils
Key terms for this diagnosis- Marocytosis with hypersegmented neutrophils
B12 deficiency
liver disease
if all cell lines down- aplastic anemia
name all 3 micocytic anemia’s
- Iron deficiency
- Thalassemia
- lead poisoning
What is a small dense basophilic RBC inclusion
Howell-Jolly Bodies
Key terms for this diagnosis- Howell-Jolley Bodies
Megaloblastic anemias
hypersplenism
Severe hemolytic anemia
Denatured Hgb in cells
Heniz cells
Key terms for this diagnosis-Heniz cells
G6PD
Thalassemia
deformity in cell due to phagocyte removal of denated hgb
Bite cell
Key terms for this diagnosis- Bite cells
- same as Heniz bodies because a bite cell is a Heniz body the hgb just got removed
G6PD
Thalassemia
Key terms for this diagnosis- schistocytes and keratocytes
Mechanical RBC damage in small vessles
microangiopathic hemolytic anemia
like TTP, HUS, DIC
Key terms for this diagnosis- Target cells
Severe Iron defiency
Sickle cell
thalassemia
Hgb S or C
Key terms for this diagnosis-Tear shaped cells
Thalassemia
hemolytic anemia
Key terms for this diagnosis-Basophilic Stippling
Lead poisoning
sideroblastic anemia- heavy metal posions
Key terms for this diagnosis- Echinocytes
aka burr cells
Uremia
Pyruvate kinase deficiency
Key terms for this diagnosis- Acanthocytes
Spur cells
liver disease like alcoholics
renal disease
autoimmune hemolytic anemia
Key terms for this diagnosis- Reticulocytes
Seen with blood loss
hemolytic anemia
30-40% of WBC’s
what is their function
Lymphocytes
viral infections
Key terms for this diagnosis- atypical lymphocytes
infectious mononucleosis- EBV
40-70% of WBC, and are multilobular
Neutrophils
infections, steroid use
reaction to gram neg sepsis
When are bands seen ?
Acute bacterial infection
when would basophils be increased
hypersentitivy reaction
when would monocytes be increased
viral infections
when would esoinophils be increased
parasitic infections
allergic reactions
an increase transferrin and increased TIBC
would be what disorder ?
Iron defiency
an decreased transferrin and decreased TIBC
would be what disorder ?
anemia of chronic diease
Ferritin is what?
Stored Iron
Decreased Ferritin would be what disorder?
Iron defiency - the body is using the stores first
an increased ferritin would be what disorder?
anemia of chronic disease
Is haptoglobin increased or decreased in iron deficency
it is decreased- it binds free hemoglobin
to reduce iron availability for microbes
seen in hemolytic anemias
How can you determine between Iron deficiency anemia and anemia of chronic disease ?
Ferritin and TIBC levels
Iron = Ferritin decrease, TIBC increased
anemia of chronic = Ferritin increased TIBC decreased
what disease is seen with Iron overload?
Hemochromatosis
what are these doing in hemolytic anemia LDH Bilirubin Haptoglobin smear cells ?
LDH is increased- it is usually inside the cell
Increase bilirubin is increased - jaundice
Haptoglobin is decrease- it is binding free hemoglobin
Schisotyctes and reticulocytosis
what is the best diagnostic test for sickle cell ?
Electrophoresis- Hgb S
Key terms for this diagnosis- MCV <80, increase serum iron, hemoglbin eletrophoresis with normal hemoglobin rations of Hgb A, A2 and F
Alpha Thalassemia
- blood smear is usually normal this is a diagnosis of exclusion
Key terms for this diagnosis- MCV <80, increase serum iron, hemoglbin eletrophoresis with decreased Hgb A, increase Hgb A2 and Hgb F
Beta Thalassemia
Key terms for this diagnosis- episodic hemotlyic anemia with sulfa drugs, infection and oxidizing agents
G6PD
what is the difference between TTP and HUS
Both ave Thrombocytopenia, hemolytic anemia and kidney damage
TTP has neuro and fevers
HUS more likely in children and to cause kidney involvement
Key terms for this anemia- pallor, glossitis, stomatitis, peripheral neruopathy, weakness, atxasia, decrease deep tendon reflexes
Macrocytic anemia
B12 deficiency
folate is more common but does not have neruo s/s
Homocysteine
Methoylmalonic acid
are see in what disorder?
Increased or decreased?
B12 deficiency
Homocysteine increased
Methoylmalonic acid increased
what is the test for pernicious anemia
schilling test
Treatment for B12 deficiency
- IM B12
watch for hypokalemia
What will happen if you give a B12 deficiency patient Folate therapy treatment without B12 replacement?
Neuro symptoms will worsen
what are other reasons for Macrocytic anemias besides B12 and folate
Alcohol abuse
liver disease
hypothyroidism
what is the most common cause of Iron Deficiency anemia
Bleeding
menstration
colon cancer
parastic hook worms
what are s/s of Iron Deficiency anemia
pagophagia- ice craving
pica
angular cheilitis
koilonychia
Key terms for this diagnosis- iron deficiency with difficulty swallowing, esophageal webs and atrophic glossitis
Iron Deficiency anemia
Plummer Vinson syndrome
Labs for Iron Deficiency anemia MCV Serum Iron Ferritin TIBC RDW HBG/HCT Transferrin saturation %
MCV - low Serum Iron- low Ferritin- low TIBC- increase RDW - increase HBG/HCT- low Transferrin saturation decrease%
Treatment for Iron Deficiency anemia
Iron pills
can cause constipation so go slow
Key terms for this diagnosis- Microcytic anemia with normal iron levels
Alpha or Beta Thalassemia
key terms for this diangosis - severe anemia, heniz sisbodies, hepatospleomegaly, microcytic hemolytic anemia, fonrtal skull and maxialla overgrowth, bone fractures and gallstones
Alpha T intermedia (Hgb H diesease)
- - / - A
key terms for this diagnosis- associated with stillbirth or death soon after from high output failure
Alpha thalassemia major- hydrops fetalis
- - / - -
how can you tell between alpha and beta thalassemia
the electrophoesis
alpha has normal Hgb ratios
Treatment for severe alpha thalassemia
- folate
- blood transufsions weekly
- iron chelation agents- defroxamine
- bone marrow transplant
what is Defroxamine for?
prevents iron overload and removes excess iron form diseases that need transfusions like
thalassemai’s
what races is thalassemia most common in ?
alpha- asain, Mediterranean (greek, italian)
beta- Mediterranea (greek, italian)
what is the most common type of beta thalassemia
B- Minor only one gene is defective
when you dont make the beta your will make more alpha
What is Cooley’s Anemia
beta thalassemia Major both B genes are mutated
leading to more alpha
Key terms for this diagnosis- At birth child has no s/s of anemia, at 6 months = jaundice, dyspena, pallor and osteopenia frontal bossing, iron overload and gall stones
beta thalassemia Major - Cooley’s anemia
patients are normal at birth because they have fetal hgb F making up for the missing beta chains
What will the eletrophoresis of Beta thalassemia show?
Minor= HgbA2 and or Hgb F increased (no beta)
Major= HgbA2 and 90% Hgb F
little or no hgb A
Treatment for Beta thalassemia
- minor- no treatment needed
- major- transusions weekly
- Deferoxamine for chelation therapy
- bone marrow transplant
Labs for this diagnosis MCV- Low RDW- Normal Ferritin- Normal Serum Iron- Normal Hgb- normal Hgb A, A2 and F
Alpha thalassemia
*remember their iron stores are not affected
Labs for this diagnosis MCV- Low RDW- Normal Ferritin- Normal Serum Iron- Normal Hgb- increased Hgb A, A2 and F
Beta thalassemia
- remember their iron stores are not affected
- remember that the Hgb beta are not present so they get replaced with other Hgb like A, A2 and F
Key terms for this diangosis- child with abd pain, consitpation, numbness tingling, ataxia, fatigue, learning disabilities, coma and shock
Lead poisoning- sideroblastic anemia
what can you seen on x-ray of lead posioning
Lead lines
linear hyperdensities of metaphyseal plates
in adults can be in their gums
What are the 3 normocytic anemias
- anemia of chronic disease
- G6PD
- Sickle cell
key terms for this diagnosis- normocytic anemia, with increased ferritin, decreased TIBC and decreased iron
anemia of chronic disease
Treatment for anemia of chronic disease
treat underlying cause
if its kidney give them erythropoietin
what is an X-linked recessive disorder primary affecting males mostly African Americans
G6PD
what is G6PD in the body for?
Glucose 6 phosphate dehydrogenase is a enzyme that protects against oxidation stress
if oxidized it gets turned into methemoglobin which doesn’t carry oxygen well
hgb on the cell gets denatured into heniz bodies
and are destroy by the spleen
leading to hemolytic anemia
what is an episode of G6PD s/s ?
back or abd pain with anemia
jaundice and splenomegaly
can lead to hemolytic crisis
rbc’s will rupture under oxidative stress
what can cause oxidative stress?
Infection- DKA, pneumonia Fava beans Sulfa drugs- bactrum antimalarials meythlene blue fluoroquinoloes nitrofurantoin asprin dapsone
Treatment for G6PD
usually self limiting
avoid whatever caused it
iron supplements
blood transfusions if severe
What is an autosomal recessive genetic disorder of Hgb SS. Valine is subsituted for what?
Sickle cells
Valine in place of Glutamic Acid on beta chain
what is one benefit of Sickle cell trait?
AA are resistant to plasmodium falciparum - malaria
what are some s/s of sickle cell disease
sickle under hypoxic conditions cause micro thrombosis hemolytic anemia hematuria painful occlusive crises
what is the most common presenting symptoms in infants with sickle cell
Dactylitis - swelling of fingers and toes
Hgb SS replaces fetal hemoglobin
What are some infections seen with sickle cell
- osteomylitis- salmonella
- risk of encasulated organism like S. pneumonia, H.flu
- Aplastic crisis with parvovirus B19
What are signs of microthrombosis or infractions with sickle cell disease
1. necrossi of the bones - humeral head H-shaped vertebrae 2. acute splenomegaly- clots are getting suck in organs 3. Skin ulcers on the tibia* 4. Stroke in a child
what are sickle cells crises caused by?
what happens?
Cells are fragile and sickle under 'stress' leading to micro infractions in ograns triggers- cold weather, hypoxia, infeciton, dehydration, alcohol, pregnancy acute onset of pain acute chest syndrome back abd pain bone pain priapism***
what will show with sickle cell hemoglobin eletrophoresis
Hgb S increased Hgb F increased NO Hgb A low Hgb and Hct on smear- target cells and sickled cells Howell-jolly bodies- if not spleen
Treatment for Sickle cell disease
- pain control - Mepridine (Demerol)
- Iv hydration
- Oxygen
- Hydroxyurea
- +/- transfusions
- Stem cell transplant only possible cure - serious side affects
Hydroxyurea is used to treat what?
sickle cell disease with painful crisis
reduces the frequency of painful crisis
What is important to do in children with sickle cell ?
Immunizaitons !!! SHIN! S. pneumonia H. Influenza Inlfuenzae B and N menigococcus any encapsulated organisms
Key terms for this diagnosis- autosomal dominant intrinsic hemolytic anemia causing a defect RBC membrane and increase cell fragility
Hereditary Spherocytosis
key terms for this diagnosis- + osmotic fragility test with negative coombs
Hereditary Spherocytosis
Treatment for Hereditary Spherocytosis
folate- helps stablize the membrane
spelenectomy in severe disease
key terms for this diagnosis- Antibodies to own RBC’s surface leading to destruction by macrophages and the spleen.
Autoimmune hemolytic anemia - AIHA
What other disorders are common with Autoimmune hemolytic anemia - AIHA
Systemic Lupus*
Rheumatoid arthritis
Chronic lymphocytic leukemia
key terms for this diangosis- IgM Antibodies, Direct coombs tests +, microspherocytes
Autoimmune hemolytic anemia - AIHA
Treatment for Autoimmune hemolytic anemia - AIHA
- warm steroids
- Splenectomy
- avoid cold exposure
Key terms for this diagnosis- rare aquired stem cell mutation that activates complemnt destruction leading to thrombosis. Dark cola-colored urine during the night or early morning with venous clots
paroxysmal notctural hemoglobinuria
What type of anemia is paroxysmal notctural hemoglobinuria
Hemolytic anemia
the protein that is suppose to protect it from complement breakdown is missing
so it gets SLICED!
what is the best test for paroxysmal notctural hemoglobinuria
Flow Cytometry
will be osmotic fragility -
coombs -
Treatment for paroxysmal notctural hemoglobinuria
- Eculizumab
2. steroids
