B.M. Failure and Myeloproliferative Disorders
what are medications that cause Aplastic Anemia (4)
chloramphenicol
carbamazepine
sulfa drugs
gold
A child with Down syndrome can get what two disorders.
AML
ALL
pain with alcohol intake and RS cells with separate nuclear lobes (owls eyes) is associated with which disorder
Hodgkins Lymphoma
what makes hemophilia C different from A,B, and acquired hemophilia
Hemophilia C can effect both women and men and is more common is Ashkenazi Jews
VTE, cerebral venous thrombosis, and recurrent pregnancy are clinical presentations of ___________
Factor V Leiden Mutation
Disorder that causes RBC to appear as ringed sideroblast
What is Myelodysplasias
what is an important variant of AML
APL (Acute Promyelocytic Leukemia)
although there is not a cure for Waldenstrom Mcroglobulinemia what would be treatments for hyper viscosity syndrome and cytopenias
hyper viscosity syndrome- plasmapheresis
cytopenias- chemotherapy
Distinguish the three different types of Von Willebrand disease
type 1: most common, autosomal dominant, quantitative deficiency, mild bleeding
type 2: qualitative deficiency with 4 subtypes
type 3: most rare and severe, autosomal recessive, presents with factor VIII deficiency
Heparin resistance can occur in _________________
and
warfarin induces skin necrosis in patients with __________________
Antithrombin III deficiency
and
Protein C&S deficiency
name the clinical presentation in Essential Thrombocythemia
Thrombotic tendencies: transient visual deficits, CVA, MI, DVT/PE
and Erythromelalgia
CML is often asymptomatic but when its not what are the 3 clinical presentations?
Fatigue
abdominal pain
bleeding
what RBC membrane and cytoskeleton proteins are missing when a patient has Hereditary Spherocytosis
spectrin and ankyrin
what two disorders both have:
Decreased platelet count on CBC
Evidence of hemolysis
Elevated creatinine
Peripheral smear with schistocytes
TTP and HUS
what disorder can produce a false positive VDRL test?
explain the Patho for this disorder
Antiphospholipid syndrome involves autoantibodies against specific membrane and plasma phospholipids, which when bound, promote the initiation of the coagulation cascade.
Aquagenic pruritus (itching when in contact with warm water), Peptic Ulcer disease, and Gout are potential symptoms and signs of which disorder?
What is Polycythemia Vera
what will you see on a CBC and Peripheral Smear for a patient with CLL
CBC: profound leukocytosis in the form of lymphocytosis
Peripheral smear: smudge cells
what is the difference between warm and cold AIHA treatments
warm: 1st line glucocorticoids 2nd line splenectomy 3rd line rituximab
cold : 1st and only rituximab
what lab finding would you expect from a patient with DIC
Prolonged PT/aPTT
hypofibrinogenemia
elevated INR
elevated D- Dimer
declining Hgb/ PLTs
schistocytes
name the 4 types of transfusion complications
hypocalcemia
allergic reaction
acute hemolytic reactions
delayed hemolytic reaction
A 38 y/o female arrives to the emergency room in a wheel chair complaining of headache, facial color abnormalities, blurry vision and complaining that are legs are burning which is why she is in a wheel chair. her CBC shows an elevated RBC, low EPO, hypercellularity on her bone barrow biopsy, and a JAK 2 mutation on her qualitative genetic analysis. what combination of drugs would you give her for treatment? (2 possible answers)
Low dose Aspirin and Peg IFN (because she is under 40)
Low dose aspirin and Ruxolitnib
what is the clinical presentation of ALL in a Child (7)
Unexplained Cytopenias
fatigue
infection
easy or spontaneous bruising/bleeding
constitutional symptoms
bone pain
hepatomegaly or splenomegaly
name medications, acute illnesses and foods that can contribute to the cause of G6PD deficiency
Meds: sulfa drugs and antimalarial drugs
Illness: infection, DKA
Foods: Fava beens
what are 1st, 2nd, and 3rd line treatment for ITP
1st line: dexamethasone & IVIG
2nd: prolonged steroid taper & rituximab
3rd: splenectomy & TPO
what are the 1st and 2nd line treatment for Hemochromatosis
1st therapeutic phlebotomies - indicated for HFE diagnosed patients when ferritin > 500 OR evidence of organ damage due to iron deposits.
2nd iron chelating if patient can not tolerate phlebotomy.