Anemia practice questions (if ya dont like em, keep it to yaself) put it on shuffle. Flashcards
A 6 year old child who is currently admitted to the hospital with pneumonia has now developed jaundice. physical exam reveals splenomegaly and a CT of her abdomen reveals gallstones. Her mother reports she has never had this illness before. there are two possible illnesses that line up with this history, what are they? what tests could you run to differentiate these and how would the tests present differently in each illness? How would you treat these?
sickle cell anemia and spherocytosis.
could do a CBC
sickle cell: ^ retic, ^WBC, decreased Hgb/Hct.
Spherocytosis: decreased Hgb/Hct, ^retic, ^MCHC.
Peripheral smear:
Sickle Cell: target cells, howell jolly inclusion bodies, sickle cells
Spherocytosis: spherocytes.
Treatment:
Sickle cell: Hydrate, Oxygenate, Pain manage. possible exchange transfusion. consider splenectomy.
Spherocytosis: consider transfusions (could use EPO to avoid) consider splenectomy.
A patient has the following lab values:
Hgb/Hct: decreased
retic: increased
MCHC: increased
MCV:normal
WBC:normal
RBC:normal
Platelets:normal
Coombs: negative
what possible illness is this patient suffering from
spherocytosis
ONLY disease that causes ^ MCHC
the peripheral smear shows: microcytic hypochromic RBCs, target cells and poikilocytosis.
the lab findings are:
Hgb/Hct: decreased
retic: increased
MCHC: normal
MCH: decreased
MCV: decreased
WBC:normal
RBC: increased
Platelets: normal
there are two possible illnesses that line up with these findings, what are they? what tests could you run to differentiate these and how would the tests present differently in each illness? How would you treat these?
- alpha thallasemmia (HbH disease)
- beta thallasemmia (intermedia)
could do electrophoresis to determine how much of each hemoglobin is present.
HbH: equal proportions of all hemoglobins and electrophoresis will reveal HbH band.
intermedia: increased HbA2 and HbF, decreased HbA.
treatment:
HbH: folic acid 1mg/day, avoid iron and oxidative drugs, tranfusions if needed, splenectomy.
Intermedia: avoid iron, transfusions, consider splenectomy.
which disease causes abnormal formation of protein membranes?
spherocytosis
a peripheral smear shows: hypochromic microcytic RBCs, target cells, poikilocytosis, Nucleated RBCs.
labs show:
Hgb/Hct: decreased
retic: increased
MCHC: normal
MCH: decreased
MCV: decreased
WBC:normal
RBC: increased
Platelets: normal
what is the disease? what is a likely physical exam finding that you would find on this patient?
How would you treat this patient?
beta thalassemia Major
PE: erythroid hyperplasia leading to chipmunk facies, thinning of long bones, pathological fractures and failure to thrive.
treatment:
avoid iron
splenectomy considered
LUSPATERCEPT (increases RBC production)
could do allogenic bone marrow transplant.
a peripheral smear shows: hypochromic microcytic RBCs, target cells, poikilocytosis, Nucleated RBCs.
labs show:
Hgb/Hct: decreased
retic: increased
MCHC: normal
MCH: decreased
MCV: decreased
WBC:normal
RBC: increased
Platelets: normal
what is the pathology of this disease? (tell me what happens to blood cells/how they are damaged)
beta thalassemia major
Pathology: Increased numbers of microcytic hypochromic RBCs. Excess alpha chains precipitate causing damage to RBC cell membranes and hemolysis in spleen, marrow and liver. Also leads to inclusion bodies which damage erythroid marrow precursors, and surviving RBCs have inclusion bodies which cause shortened life span.
a teenage patient presents to you with complaints of anxiety and palpitations. When you take a closer look you realize that she has a hyperdynamic precordium and her fingers are swollen and tender. she reports that she has been extremely stressed lately due to school, and has been having some slight pain in her right thigh. based off of these physical findings, what kind of anemia could this patient possibly have? what is the cause and pathology of this disease?
i know this is rough just bear with me lol
sickle cell anemia.
Cause: Autosomal recessive disease. Abnormal substitution of amino acid in beta chain causing “beta S” chain. Causes HgbS to form.
Pathology: Hbg causes abnormally shaped RBCs (sickled cells) which causes vaso occlusive episodes because they stick to the endothelium.
A peripheral smear shows bite cells, blister cells, and heinz bodies.
what is the cause and pathology of this anemia?
G6PD deficiency
Cause: X-liked recessive genetic inheritance. This causes a defect in Gluc-6-phosphate dehydrogenase enzyme which leads to RBCs being broken down prematurely.
Pathology: RBCs are more susceptible to oxidative stress which causes heinz bodies to precipitate around the cell and break down the membrane of the RBC. RBCs can also spontaneously lyse within the vessels. The RBCs w heinz bodies are eaten by the spleen
The labs for a patient are as follows
Hgb/Hct: decreased
retic: increased
MCHC:normal
MCH:normal
MCV:normal
WBC:normal
RBC:normal
Platelets:normal
the physical exam of this patient shows jaundice, dark urine and abdominal tenderness. but NO hepatosplenomegaly.
What is the likely disease and what are the treatment options?
G6PD deficiency
treatment options;
preventative: avoidance of triggers, avoidance of oxidative drugs, genetic counseling
Therapeutic: Folic acid 1mg/day, removal of offending agent, rarely transfusions are needed.
remember this is caused by triggers such as medications (NSAIDS, ABX, quinolones), foods (fava beans, soy, red wine, blueberries) and infections
a patient presents with sudden onset severe anemia. she has fatigue, jaundice and splenomegaly. her labs are as follows:
Hgb/Hct: Very Low
retic: increased
MCHC: normal
MCH: normal
MCV:normal
WBC: normal
RBC:decreased
Platelets: normal or decreased
What is the cause and pathology of this patient’s anemia?
Autoimmune hemolytic anemia:
Cause: antibody forms against surface RBC antigens. 50% unknown, 50% caused by AI dx like lupus.
Pathology: RBC is “tagged” by immune system
First method - spleen sends splenic macrophages to eat the RBCs - leads to spherocyte
Second method -complement tags them and liver sends kupffer cells to eat them
Third method - IgM helps facilitate MAC induced direct intravascular hemolysis.
a patient presents and her peripheral smear shows: microspherocytosis and polychromataphils/reticulocytes.
what is this patients illness and what are their possible treatments?
Autoimmune hemolytic anemia:
treatments:
Immunosuppression - prednisone 1-2 mg/kg daily.
Splenectomy in severe cases
Treatment of any underlying comorbidities/disorders
Cold avoidance is cold AIHA
Transfusions often required due to severity.
A mother with type O- blood is pregnant with a baby who is type A+. what disease is this patient at risk for? what treatment options are available for these patients? What PE findings will be found in the baby when they are born?
Hemolytic disease of the newborn
before birth: intrauterine fetal transfusion, early induction of labor, maternal plasma exchange, could also use RhoGAM.
after birth: transfusion and supportive care.
baby will be born: jaundiced, hepatosplenomegaly, severe anemia, heart failure, and edema.
a patient has a deficit in complement regulating cell membrane proteins CD55 and CD59 due to a genetic defect that leads to lysis of RBCs.
what type of labs would be helpful in diagnosing this patient if they presented to the hospital with severe anemia.
this is paroxysmal nocturnal hemoglobinuria.
urine hemosiderin (positive)
Flow cytometry (positive)
a patient presented with esophageal spasms and erectile dysfunction, after labs were obtained it was found that he was anemic and has a thrombosis in the liver. What type of anemia does this patients likely have and what are the treatment options for this patient?
this is paroxysmal nocturnal hemoglobinuria.
only definitive is allogenic hematopoietic stem cell transplant.
for thrombosis could use eculizumab (monoclonal antibody against C5) and supportive methods.
lab findings show:
RBC: decreased
Retic: Decreased
MCH:normal
MCHC: normal
MCV: normal
WBC:decreased
Platelets: decreased
bone marrow biopsy shows hypocellular aspirate; limited or no hematopoietic precursors
what is the likely diagnosis? what are the treatment options? (consider supportive, mild, severe)
aplastic anemia
supportive: RBC and Platelet transfusion. Antimicrobials
Mild:
-multifactorial: eltrombopag/promacta
-erythropoietic: epoetin/darbepoetin
-myeloid: filgastram/sargramostim
Severe: bone marrow transplant - <40 w fully HLA-matched sibling donor
immunosuppression vai: equine ATG+cyclosporine+corticosteroid+/-eltrombopag
