AWESOME HEME Flashcards
What is the most sensitive assay for iron deficiency anemia?
Ratio of transferrin receptor to log ferritin (transferrin receptor index); >2.0 is iron deficiency and <1.0 is anemia of inflammation
Verotoxin can cause a TMA and is produced by what organism?
E. coli 0157:H7 i.e. basically HUS
What is a normal serum ferritin? Levels greater than ______ essentially rule out iron deficiency?
20; 100
What should you think of in a patient with numbness s/p colonoscopy with numbness on the L side of the body that resolves in 15 min
Could be air embolus that traveled through the colon venous circulation and crossed a PFO
If a patient has hgb of 10, MCV of 85, and t sat of 12% with ferritin of 120 what will a bone marrow biopsy show?
This is anemia of inflammation so there will be lots of iron in the macrophages but lower amounts of iron in the RBC precursors
In which type of AIHA are steroids useful?
Warm AIHA (IgG +/- C3)- removed by spleen whereas cold removed more by liver
What is the only medication shown to decrease mortality in RA?
MTX
What is the process from ALA to heme?
ALA–>Porphobilinogen–>HMB–>uroporphyrogen–>coproporphryogen–>protoporphyrin–> heme; Acute intermittent porphyria d/t decreased PBG deaminase with elevation of PBG (i.e. Dx with spot urine porphobilinogen) and porphyria cutanea tarda due to uroporphyrogen decarboxylase wiht elevated levels of uroporphyrogen
What is present in hemoglobin A?
Normal adult hgb- 2 alpha chains and 2 beta chains
The primary tx for post-transfusion purpura is _______
IVIG (and for CLL, ITP, and GBS)
What should be thought of in anemias with MCV >110?
Likelly B12 or Folate deficiency; of note when there are ridiculous MCVs like 140 should think of cold agglutinin dz with agglutination being counted as the MCV. There are other causes of macrocytosis but MCV >110 mostly just folate and B12 def (i.e. aplastic anemia and MDS likely not that high)
What is a good way to differentiate B12 deficiency and vitamin B6 deficiency anemia?
High MCV in B12 def, also the homocysteine will be high in both so ignore it
An african american man with SC disease presents with right hip pain. No hx of recent trauma and XR of hip shows diffuse articular sclerosis and patches of decalcification what is the Dx?
AVN of the femoral head; this MC occurs in SC dz (also may have splenomegaly and retinal infarcts)
What disorder can cause a smooth and bald tongue with cheilosis and posterior column disease?
B12 def; this is subacute combined degeneration of the cord (similar stuff seen in tertiary syphilis with tabes dorsalis)
75 F with forgetfulness and positive Romberg sign. B12 is 310, hgb is 13 with MCV of 103, wtd next?
Check MMA level (borderline low B12 –> check MMA); folate will improve anemia but will not correct neuropsychiatric sx
Which heparinoid can be used for patients with hyperkalemia?
Fondaparinux
What is the enzyme deficiency in porphyria cutanea tarda?
Uroporphyrogen decarboxylase which leads to elevated levels of uroporphyrogen; of note anything messing with hepatocyte metabolism can cause this deficiency hence relationship to HCV
What is present in hemoglobin F?
2 alpha chains and 2 gamma chains
A pt with unstable angina is started on IV nitrates or post-EGD or post-bronchoscopy turns cyanotic and SOB ABG shows PaO2 of 96 with pulse ox of 85% DX?
Methemoglobinemia (benzocaine spray or nitrates can cause this (ferrous–>ferric)); also the mismatch in PaO2 to pulse ox = methemoglobinemia
A hemoglobin electrophoresis with mostly HbA but also with HbS and maybe a bit of HbA2 and HbF is suggestive of this ________
Sickle cell trait as true SS disease would not have any HbA (recall, trait can have splenic sequestration crisis and SS disease cannot)
Which type of AIHA will occur in CLL or a lymphoma?
Anti-IgM with IgM and C3+ and destruction largely in liver
A patient with sickle cell trait with LUQ pain and an enlarging spleen and dropping hb with BP 100/80 supine and 80 when standing? What is the Dx? What is the next step and prognosis?
Splenic Sequestration crisis (recall that this can only occur in an adult with trait as SS will have autoinfarcted the spleen in childhood); fluids the pRBCs for volume expansion; not Tx with surgery as most resolve on their own
Which countries have more alpha thalassemia? Which have more beta?
Alpha- Laos, Cambodia, Vietnam (SE Asia); Beta- Italy, Greece etc
A patient with membranous nephropathy with PE and negative duplex US of the LE still warrants investigation with ________
Renal vein duplex as the renal vein thrombosis can still cause PE
Which breast cancer drugs can predispose to osteoporosis?
Aromatase Inhibitors
If a patient is an adult with sickle cells (drepanocytes) seen on peripheral smear and has a splenic sequestration crisis then what is the underlying diagnosis?
Sickle Cell Trait as a true SS dz would have autosplenectomized by then already
Regarding folic acid def, B12 def, and B6/pyridoxine def, what will be the results of homocysteine and MMA levels?
Folic acid and B6 will have only elevations of homocysteine; B12 will have elevation of both homocysteine and MM
What is the etiology of acute intermittent porphyria?
Decreased activity of porphobilinogen deaminase with elevated levels of porphobilinogen PBG causing neuropsychiatric sx
Which has elevated PT and ammonia–HELLP or Acute Fatty Liver of Pregnancy?
Acute Fatty Liver of Pregnancy
What effect does polycythemia vera have on the HbA1C?
Falsely low because there are so many RBCs relative to the amount usually in the assay
What is the leading cause of mortality in sickle cell patients?
Acute Chest Syndrome (IV abx, prbc if severe anemia, and O2 if hypoxic) if hypoxia persists despite O2 and pRBCs then exchange xfusion
What is likely to be the best Tx of Burr Cell anemia?
Erythropoietin as the etiology is due to decreased EPO production