Heme/Onc KPs Flashcards
leads to disease control in 70% of adult patients with aplastic anemia?
cyclosporine and antithymocyte globulin
Immunosuppression
(9;22) gene translocation suggests? Treatment? Monitor this while on treatment?
CML;
TKR inhibitors (target BCR-ABL). Can prolong QT interval
Initial therapy for polycythemia vera?
When to add additonal therapy? (and what?)
ASA + phlebotomy
age>60 or high VTE risk - add hydroxyurea
General therapy for essential thrombocytopenia? Next med to add?
Observation
Platelet-lowering therapy (hydroxyura v anagrelide) if high risk
Treatment for primary myelofibrosis? unless?
Palliative (unless young - then HCT)
hypereosinophilic syndrome - treatment? If fails?
steroids; imatinib
defining clinical clue to promyelocytic leukemia? characterized by what mutation? Treatment?
DIC
t(15;17)
all-trans retinoic acid
Philadelphia chromosome–positive acute lymphoblastic leukemia can be treated with?
dasatinib (TKR)
most sensitive test for detecting monoclonal FLC gammopathies?
serum free LCs
Monoclonal gammopathy of undetermined significance is defined as?
If asymptomatic but otherwise does not meet this conditions?
M protein level less than 3 g/dL (or less than 500 mg/24 hr of urinary monoclonal free light chains), clonal plasma cells comprising less than 10% of the bone marrow cellularity, and the absence of plasma cell dyscrasia–related signs or symptoms
Smoldering multiple myeloma
Management of most patients with smoldering multiple myeloma?
CBC, Ca, Cr and M-protein assessment every 3-6 months
Best therapy for MM?
auto-SCT + melphalan after initial induction therapy
Proteins in AL? AA? Hereditary? Age-related? Dialysis related?
- Monoclonal free K/L LCs
- transthyretin (TTR)
- Serum amyloid A
- TTR
- B2 microglobulin
Assocaited for AL v AA amyloid?
AL - Plasma cell dyscrasias (MGUS, multiple myeloma), Waldenström
AA -Rheumatoid arthritis, inflammatory bowel disease, familial Mediterranean fever, chronic infection
Amyloid treated with SCT?
AL
Concern about Waldenstrom? Telltale symptoms?
hyperviscosity - headache, vision/hearing changes, dizziness, AMS
Dilated retinal veins, papilledema, and flame hemorrhages - think?
hyperviscosity syndromes (Waldenström or Type I cryoglobulinemia etc)
Normal trasferrin sat? Normal Ferritin?
<15; <12
Anemia in CKD - no diagnostic role for measuring?
erythropoietin
B12 v folate deficiency?
Methylmalonic acid and homocysteine are oth elevated
normal MMA, high HC
Before starting folate treatment, need to rule out?
concomittant B12 deficiency (otherwise the increased hematopoisis can make patients have NS injury)
chronic hemolytic anemia - need what supplementation?
folic acid
bite cells and Heinz bodies?- suggest?
G6PD
β-thalassemia v a-thalassemia - hemoglobin concentrations?
increased hemoglobin A2 levels and fetal hemoglobin levels vs normal
disease-altering strategies in sickle cell disease?
HCT, transfusions, hydoxyurea
mainstay treatments for warm autoimmune hemolytic anemia? Cold agglutinin disease?
Steroids
Coid avoidance (rituximab is fails)
cause of cold agglutinin disease?
Pathogenic IgM molecules -> bind colder temperatures, typically in the fingers, toes, and nose
ID causes of hemolytic anemia?
Malaria Plasmodium,
Babesia microti,
Clostridium perfringens, and
Bartonella bacilliformis
Atypical hemolytic uremic syndrome? Effectively treated by?
congenital syndrome affecting complement activation (not preceeded by diarrheal illness)
eculizumab
diagnostic test for ITP? Requires therapy when?
none; PLT<30 or bleeding
heparin-induced thrombocytopenia - gold stardard for diagnosis?
serotonin release assay
Diagnosis of von Willebrand disease?
- von Willebrand factor antigen and activity <30%
- low/normal factor VIII levels
- Normal PT
- High/normal PTT
supranormal factor VIII levels seen in?
liver disease
Never give alloimmunized patients?
erythrocytes containing antigens to which they are alloimmunized, even if repeat alloantibody screening is negative
resh frozen plasma is ineffective for treating coagulopathies with an INR under?
1.85
Inactivated 4-factor prothrombin complex concentrate contains? Give when?
II, VII, IX, X
Warfarin associated bleeding
elayed hemolytic transfusion reactions occur? present with?
2-10 days after exposure
fever, jaundice, and worsening anemia
Patients at high risk for anaphylactic transfusion reactions?
IgA deficiency
transfusion-associated graft-verus-host disease - what is it? who is affected? Window of onset? Signs and symptoms? Prevention?
donor lymphocytes generate an immune response to recipient tissues
immunocompromised and unable to destroy the donor lymphocytes or in immunocompetent patients who are heterozygous for an HLA haplotype for which the donor is homozygous
up to 30 days after transfusion
Rash, jaundice, n/v/d, pancytopenia
Irradiation
superficial venous thrombophlebitis treatment?
symptomatic therapy
most common risk factor for Budd-Chiari syndrome?
myeloproliferative neoplasm (polycythemia vera, essential thrombocythemia, and primary myelofibrosis)
diagnostic tests of choice for cerebral and sinus vein thrombosis?
Treatment?
CT venography and magnetic resonance venography
3 to 6 months if cerebral and sinus vein thrombosis was associated with a transient risk factor, 6 to 12 months if the event was unexplained and no higher risk thrombophilia has been detected, and long term if a higher risk thrombophilia is detected or the event is recurrent
reverses anticoagulation with unfractionated heparin?
protamine
Avoid LMWH if? Also avoid this anticoagulant?
CrCl<30; Fondaparinux
patients taking warfarin with fluctuating INRs, daily supplementation this can stabilize the INR.
vitamin K
When to evaluate gestational anemia?
Hgb<11 in first trimester
Hgb<10 in 2nd/3rd trimesters
SIckle cell meds that must be discontinued in pregnancy?
Hydroxyurea, ACE inhibitors, and iron chelation therapy
Goal PLT count in last month of pregnancy?
50 in case C-section is needed
Highest risk of VTE during preggnancy is when?
6 weeks postpartum
