Hematology Flashcards
What genetic type of alpha thal has high reticulocytes?
3 gene deletion
when 4 genes are deleted in alpha thal what can you expect?
gamma-4 tetrads or hgb bart; CHF causes death in utero
iron chelators needed for excessive transfusion in patients with Beta thalassemia?
Oral iron chelators: deferiprone and deferasirox
Goats milk, psoriasis, phenytoin and sulfa drugs and methotrexate use can all cause a deficiency in?
Folate
What is a complication of B12 or Folate replacement? why?
Hypokalemia: extremely rapid cell production in bone marrow causes hypokalemia.
Why might pancreatic enzyme deficiency cause B12 deficiency?
Pancreatic enzymes are needed to remove B12 from R-protein so that it can bind intrinsic factor and be reabsorbed
Genetic cause of sickle cell?
point mutation at position 6 of beta globin chain. valine replaces glutamic acid
2 things that lower mortality in sickle cell disease?
hydroxyurea in prevention
antibiotics with fever
Morulae are?
seen inside of neutrophils in Ehrlichia infection
how does hydroxyurea benefit sickle cell patients?
reduces recurrence of sickle cell crises by increasing hemoglobin F
When is exchange transfusion indicated for sickle cell disease?
Acute chest syndrome, priapism, stroke, retinal infarct
First clue to parvovirus in patient with sickle cell?
sudden drop in reticulocyte count
What is the first test you should order in a patient with sickle cell with a sudden drop in Hct?
Reticulocyte count (usually retic count is elevated in sickle cell due to chronic hemolysis)
Suspect parvo
most accurate test for parvo infection? best initial therapy?
PCR for DNA
IV immunoglobulin
2 benign manifestations of sickle cell trait?
isothenuria (inability concentrate urine)
hematuria
Most likely diagnosis for recurrent episodes of hemolysis, intermittent jaundice, splenomegaly, family history of anemia/hemolysis, bili gallstones
hereditary spherocytosis
MCHC is elevated in?
hereditary spherocytosis
Treatment for hereditary spherocytosis?
splenectomy
folic acid
What 2 diseases are associated with spherocytes?
HS and autoimmune hemolysis
Why doesn’t a smear show fragmented cells in autoimmune hemolysis?
red cell destruction occurs in spleen or liver. not blood vessel
best initial therapy for autoimmune hemolysis?
glucocorticoids
recurrent episodes of autoimmune hemolysis respond to? severe, acute hemolysis that doesn’t respond to prednisone should be treated with?
splenectomy
IVIG
Alternate treatments for autoimmune hemolytic anemia to decrease steroid use if splenectomy doesn’t work?
cyclophosphamide, cyclosporine, azathioprine, mycophenolate
Cold agglutinin anemia occurs in association with what diseases?
EBV, Waldenstroms, Mycoplasma Pneumo
Treatment for cold agglutinin autoimmune hemolytic anemia?
stay warm! rituximab! plasmapharesis. cyclophosphamide, clyclosporine
most common wrong answer for treatment of cold agglutinin? what else doesn’t work to treat?
Prednisone (doesn’t work!)
splenectomy! (wrong)
Cryoglobulinemia is associated with?
Hep C, joint pain, glomerulonephritis
Best initial test for G6PD? most accurate test for G6PD after 1-2 weeks?
initial: heinz bodies and bite cells on smear
most accurate: G6PD level 1-2 weeks later
HUS and TTP are both caused by deficiency in?
ADAMTS13
What will worsen the disease in patients with TTP and HUS?
platelet transfusion
Treatment for TTP and HUS?
severe cases?
Steroids
Plasmapharesis
Deficiency in compliment regulatory proteins CD55 and 59?
PND -> leads to overactivation of complement system
most common causes of death in PND?
thrombosis of mesenteric or hepatic veins
episodic dark urine, pancytopenia, iron deficiency, clots in weird places?
PND
best test for PND diagnosis?
flow cytometry /decreased CD 55-59
method of cure for PND
bone marrow transplant
Treatment of PND?
Prednisone- initial therapy
Eculizumab- inactivates C5
aplastic anemia is?
pancytopenia of unclear etiology. autoimmune in which T cells attack patients own marrow.
Treatment for aplastic anemia
If > 50 or no matched donor: cyclosporine: inhibit T cells and antithymocyte globulin (ATG)
If < 50 and matched donor: allogeneic transplant of bone marrow
Target hct for polycythemia vera?
< 45%
Treatment for P. vera
- phlebotomy and aspirin prevent thrombosis
- hydroxyurea
- allopurinol
- antihistamine
MOA of ruxolitinib
inhibits JAK2
can be used in P. vera
essential thrombocytopenia
myelofibrosis
Platelet count in essential thrombocytosis is often >?
1 million
best initial therapy for essential thrombocytosis if patients is > 60 and has thromboses?
hydroxyurea
tear drop cells and nucleated RBCs on peripheral smear?
Myelofibrosis
Thalidomide and lenalidomide are useful in myelofibrosis treatment because?
TNF inhibitors. increase bone marrow production
A history of myelodysplastic syndrome might suggest?
acute leukemia
Acute promyelocytic leukemia is associated with what condition?
DIC
Myeloperoxidase is characteristic of?
acute myelocytic leukemia
Rasburicase MOA?
prevents tumor lysis related rise in uric acid
What therapy is important to add for ALL treatment?
Add intrathecal methotrexate!
Auer rods = ?
AML
Add ATRA for treatment with?
M3 (Promyelocytic leukemia)
Most accurate test to diagnose CML?
BCR-ABL (done by PCR or FISH) on peripheral blood
Blood smear findings in CML
persistently high WBC count all neutrophils. <5% blasts. increased basophils
Most effective cure for CML?
BMT
Best initial treatment for CML?
tyrosine kinase inhibitor
CML has the greatest likelihood of all myeloproliferative disorders to transform into…?
Acute leukemia (blast crisis)
A patient presents with symptomatic WBC count of 225,000. what is your first step in management?
Leukapharesis
5q deletion is standard abnormality in? Patients with 5q have better or worse prognosis?
Myelodysplastic syndrome
Better prognosis
5q deletion in MDS responds to what drug?
lenalidomide
Severity of MDS is based on?
number of blast cells
Pelger-Huet cells
bilobed nucleus. characteristic cells of MDS
Azacitidine significance in MDS
decreases transfusion dependence and increases survival
Richter phenomenon
conversion of CLL into a high-grade lymphoma
Smudge cell
lab artifact where fragile nucleus is crushed by a cover slip (seen in CLL)
Prophylaxis for what bug is indicated in CLL?
PCP
Treat refractory cases of CLL with?
cyclophasphamide
Mild cases of CLL in the elderly with?
chlorambucil
Severe CLL infection treat with?
IVIG
Fludarabine, cyclophosphamide, rituximab are best therapy for??
CLL
Dry tap despite hypercellularity of bone marrow suggests?
Hairy cell leukemia (B-cells with filamentous projections
Cladiribine or pentostatin are good treatments for?
Hairy cell leukemia
best initial test to diagnose non hodgkins lymphoma?
excisional biopsy
How often does NHL present in an advanced stage?
80% - 90% of the time
For advanced disease of NHL treatment is with?
R-CHOP
Hodgkin lymphoma centers around what part of body?
cervical area
Which type of hodgkin has worst prognosis?
lymphocyte depleted
which type of non-hodgkin has worst prognosis?
Burkitt and Immunoblastic
Treatment for stage III or IV hodgkin ?
ABVD
You cant use adriamycin if ejection fraction is less than?
50%
What is the most useful test to perform to determine the dosing of chemotherapy in Hodgkin lymphoma?
MUGA or nuclear ventriculogram because Adriamycin (dano and doxorubicin are cardio toxic)
Adverse effect of doxorubicin (adriamycin)
cardiotoxicity
adverse effect of vincristine
peripheral neuropathy
adverse effect of bleomycin
lung fibrosis
cisplatin adverse effect
ototoxicity and nephrotoxicity
cyclophosphamide adverse effect
hemorragic cystitis
most common presentation of mulitple myeloma?
bone pain from pathologic fractures
Reason for bone pain, breaks and hypercalcemia in multiple myeloma?
osteoclast activating factor is released
most common causes of death in multiple myeloma
renal failure and infection
greater than ____ % plasma cells defines myeloma
10 %
Why does multiple myeloma have an decreased anion gap?
IgG is cationic. cations increase chloride and bicarb levels.
Does urine dipstick detect jones protein?
no (need urine electrophoresis)
Single most accurate test to diagnose multiple myeloma?
greater than 10% plasma cells on bone marrow biopsy (most common wrong answer is SPEP).
Why isnt SPEP the best test for MM?
99% of people with M spike do not have multiple myeloma. they have MGUS
lethargy, blurry vision with vertigo, engorged blood vessels in eye, mucosal bleeding, raynauds is a common presentation for?
Waldenstrom Macroglobulinemia
Best therapy for Waldenstroms
plasmapharesis
