Hematology Flashcards by Ashley Hamel

What genetic type of alpha thal has high reticulocytes?

3 gene deletion

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when 4 genes are deleted in alpha thal what can you expect?

gamma-4 tetrads or hgb bart; CHF causes death in utero

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iron chelators needed for excessive transfusion in patients with Beta thalassemia?

Oral iron chelators: deferiprone and deferasirox

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Goats milk, psoriasis, phenytoin and sulfa drugs and methotrexate use can all cause a deficiency in?

Folate

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What is a complication of B12 or Folate replacement? why?

Hypokalemia: extremely rapid cell production in bone marrow causes hypokalemia.

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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

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Genetic cause of sickle cell?

point mutation at position 6 of beta globin chain. valine replaces glutamic acid

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2 things that lower mortality in sickle cell disease?

hydroxyurea in prevention

antibiotics with fever

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Morulae are?

seen inside of neutrophils in Ehrlichia infection

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how does hydroxyurea benefit sickle cell patients?

reduces recurrence of sickle cell crises by increasing hemoglobin F

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When is exchange transfusion indicated for sickle cell disease?

Acute chest syndrome, priapism, stroke, retinal infarct

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First clue to parvovirus in patient with sickle cell?

sudden drop in reticulocyte count

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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

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most accurate test for parvo infection? best initial therapy?

PCR for DNA

IV immunoglobulin

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2 benign manifestations of sickle cell trait?

isothenuria (inability concentrate urine)

hematuria

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Most likely diagnosis for recurrent episodes of hemolysis, intermittent jaundice, splenomegaly, family history of anemia/hemolysis, bili gallstones

hereditary spherocytosis

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MCHC is elevated in?

hereditary spherocytosis

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Treatment for hereditary spherocytosis?

splenectomy

folic acid

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What 2 diseases are associated with spherocytes?

HS and autoimmune hemolysis

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Why doesn’t a smear show fragmented cells in autoimmune hemolysis?

red cell destruction occurs in spleen or liver. not blood vessel

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best initial therapy for autoimmune hemolysis?

glucocorticoids

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recurrent episodes of autoimmune hemolysis respond to? severe, acute hemolysis that doesn’t respond to prednisone should be treated with?

splenectomy

IVIG

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Alternate treatments for autoimmune hemolytic anemia to decrease steroid use if splenectomy doesn’t work?

cyclophosphamide, cyclosporine, azathioprine, mycophenolate

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Cold agglutinin anemia occurs in association with what diseases?

EBV, Waldenstroms, Mycoplasma Pneumo

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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

1. phlebotomy and aspirin prevent thrombosis 2. hydroxyurea 3. allopurinol 4. 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

Hematology Flashcards

(97 cards)