Hematology Flashcards
What is acute lymphocytic leukemia (ALL)?
Child + lymphadenopathy + bone pain + bleeding + fever in child, bone marrow > 20% blasts in bone marrow
- population: children - most common childhood malignancy peak age 3-7
- highly responsive to chemotherapy (remission >90%)
What is chronic lymphocytic leukemia (CLL)?
middle age patient, often asymptomatic (seen on blood tests), fatigue, lymphadenopathy, splenomegaly
- population: adults - most common form of leukemia in adults - peak age 50 y/o
- diagnostic studies: SMUDGE CELLS on peripheral smear, mature lymphocytes
- treatment with observation, if lymphocytes are 100,000 or symptomatic, treat with chemotherapy
What is acute myeloid leukemia (AML)?
blasts + auer rods in adult patients
- population: adults (80%) majority of patient > 50 y/o
- anemia, thrombocytopenia, neutropenia, splenomegaly, gingival hyperplasia and leukostasis (WBC >100,000)
- aur rods and >20% blasts seen in bone marrow
What is chronic myeloid leukemia (CML)?
strikingly increased WBC count >100,000 +m hyperuricemia + adult patient (usually >50 years old)
- population: adults - patient usually > 50 y/o
- 70% asymptomatic until the patient has a plastic crisis (acute leukemia)
- diagnostic studies: philadelphia chromosome (translation of chromosome 9 and 22) - “Philadelphia CreaM cheese” splenomegaly
What is anemia of chronic disease?
a form of anemia seen in chronic infection, chronic immune activation and malignancy
-multifactorial; often with iron deficiency
How is anemia of chronic disease dx?
usually needs the presence of chronic infection, inflammation, cancer, microcytic/normocytic anemia, and values for serum transferrin receptor and serum ferritin that are between those typical for iron deficiency and sideroblastic anemia
What are the lab values for anemia of chronic disease?
normal or decreased MCV, decreased TIBC, increased ferritin (high iron stores)
- some transferrin converted to lactoferrin which can’t transport ferritin - decreased TIBC
- increased ferritin because it is not being transported to bone marrow
What are the MC causes of anemia of chronic disease?
chronic renal failures and anemia from connective tissue disorder: RA, SLE, HIV, CA, cirrhosis, chronic infection
What is anemia of renal failure?
erythropoiesis impaired because of decreased in EPO production and marrow responsiveness to EPO
-renal insufficiency + decreased serum EPO
How is anemia of renal failure dx?
normochormic/normocytic anemia initially, decresaed MCV, decrased TIBC, increased ferritin
- decreased serum erythropoietin in anemia of renal failure
- CBC and serum iron, ferriting, transferrin, transferrin receptro
What is the tx of anemia of renal failure?
treat underlying disorer, recombinant EPO and iron supplementation
-treat with EPO analog (Epogen, procrit) if Hgb < 10 and stop when Hgb >11 d/t increase chance MI/stroke
What are clotting factors?
are proteisn that interact to help the blood clot, stopping the bleeding
- there are two clotting factor disorders we should focus on
- von Willebrand Disese (vWD) - missing protein for platelt function
- the hemophilias
- hemophilia A decreased clotting factor VIII
- hemophilia B decreased clotting factor IX
What is von willebrand disease?
the von Willebrand factor is found in plasma, platelets, and walls of blood vessels
- when the factor is missing or defective, platelets cannot adhere to the vessel wall at the site of an injury
- as a result, bleeding does not stop as quickly as it should
- most common genetic bleeding disorder, autosomal dominant
- decreased von Willebrand’s factor and decreased Factor VIII
- patients may present with excessive bleeding after a cute or increased menstrual bleeding
- you can differentiate this from hemophilia by lack of hemarthrosis, small amounts of superficial bleeding, common to have bleeding with minor injury and petechiae
How do you treat von willebrand disease?
treat with DDAVP (desmopressin) or in cases of excessive bleeding a transfusion of concentrated blood clotting factors containing von Willebrand factor
What is hemophilia?
a hereditary bleeding disorder caused by a deficiency in one of two blood clotting factors: factor VIII (A) or factor IX (B)
What are the two forms of hemophilia?
- hemophilia A, which accounts for about 80% of all cases, is a deficiency in clotting factor VIII (“aight”)
- hemophilia B is a deficiency in clotting factor IX (Christmas disease)
- remember hemophilia A = “aight” and B comes after A which is factor nine
- x-linked recessive so will affect males (most of the time)
- hemarthrosis, bruising, and bleeding
- increased PTT, normal PT and platelets, with decreased factors VIII or decreased factor IX on assay
What is the tx for hemophilia?
treatment involves the replacement of factor VIII or IX
What is G6PD deficiency?
after infection or medication (oxidative stress) in an African American male (x-linked) + Heinz bodies and bite cells on smear (damaged hemoglobin - G6PD protects RBC membrane)
- hemolytic anemia
- african, middle eastern, s. asian population
- flare triggers: fava beans, antimalarials, sulfonamides
- diagnostic studies: Heinz bodies and bite cells on smear
What is the tx of G6PD deficiency?
- avoid potentially harmful drugs, monitor infection
- acute - blood transfusion
What is are the differential diagnosis of hypercoagulable state?
the differential diagnosis can be remembered with the mnemonic PVCs:
- platelets: too many (usually more than 1 million/uL) or overactive (TTP, heparin-induced thrombocytopenia (HIT), HUS, and HELLP)
- Vascular injury: from plows, trauma, or burns
- Clotting factors: anti-clotting factors protein C, protein S, or Antithrombin III deficient or not working
- Stasis and surgery
What is virchow triad?
blood stasis, hypercoagulable state, and vascular injury
What are the causes of hyper coagulable state?
- genetic causes include antithrombin III deficiency, factor V Leiden (activated protein C resistance), protein C deficiency, protein S deficiency, dysfibrinogenemia, and abnormal plasminogen
- acquired hypercoagulable states are associated with malignancy (Trousseua’s syndrome), pregnancy, nephrotic syndrome, ingestion of certain medications (especially estrogen), immobilization, myeloproliferative disease, ulcerative colititis and Crohn disease, behcet syndrome, polycythemia vera, intravascular devices, DIC, hyperlipidemia (particularly familial type II hyperbetalipoproteinemia), PNH, TTP-HUS, hyperviscosity syndrome, anticardiolipin antibodies, HIT, and antiphospholipid syndrome
- heparin therapy (unfractionated heparin (UFH) or low-molecular-wegiht heparin (LMWH) can cause HIT, causing a decrease in platelts (usually in half), followed by platelet activation causing clotting and infarction
- lupus anticoagulant, an IgM or IgG immunoglobulin is seen in 5% to 10% of patients with SLE but is more common in persons without lupus or in those taking phenothiazines
What is Factor V Leiden (MC)?
procoagulant clotting factor - amplifies the production of thrombin - clot formation
- mutated factor V resistant to breakdown by activated Protein C - results in hypercoagulability
- symptoms/phycial exam - increased DVT and PE, especially in young patients
- diagnosis with activated protein C resistance assay (factor V Leiden specific functional assay) - if positive, confirm with DNA testing, normal PT/PTT
- tx: LMWH bridge to warfarin; long term antithrombotic therapy not recommended
