Term
| at what Hb level is transfusion recommended? |
|
Definition
|
|
Term
| 1 unit of packed RBC's increases Hb and Hct by how much? |
|
Definition
Hb by 1 point
Hct by 3 points |
|
|
Term
| what is in PRBC vs FFP vs cryoprecipitate |
|
Definition
PRBC - no platelets or clotting factors - mix with NS to infuse faster.
FFP - all clotting factors, no RBC/WBC/platelets
cryoprecipitate - factor 8 and fibrinogen. used for hemophilia A, DIC, and vWD. |
|
|
Term
| 1 unit of platelets raises platelet count by how much? |
|
Definition
|
|
Term
| what is the importance of retic count in anemia? |
|
Definition
tell whether or not the body is able to respond adequately to the anemia.
Retic count > 2% = adequate bone marrow response.
Retic count < 2% = inadequate RBC production by the bone marrow |
|
|
Term
| types of microcytic anemia (3) |
|
Definition
iron deficiency (MC)
thalassemia
ring sideroblastic anemias (lead poisoning, pyridoxine deficiency, toxic effects of alcohol) |
|
|
Term
| casues of macrocytic anemia (3) |
|
Definition
nuclear defect - B12, folate deficiency
liver disease
stimulated erythropoiesis |
|
|
Term
| causes of normocytic anemia (5) |
|
Definition
aplastic
bone marrow fibrosis
tumor
anemia of chronic disease
renal failure |
|
|
Term
|
Definition
|
|
Term
| lab results in iron deficiency anemia |
|
Definition
decreased Fe
increaed TIBC
elevated transferrin levels
decreased serum iron
microcytic hypochromic RBC's on smear |
|
|
Term
| gold standard for dx of iron deficiency anemia |
|
Definition
| bone marrow biopsy (rarely performed) |
|
|
Term
| methods of iron replacement |
|
Definition
oral = ferrous sulfate
IV/IM = iron dextran |
|
|
Term
| who is beta thalassemia most common in? |
|
Definition
| people of Mediterranean, Middle Eastern, and Indian ancestry. |
|
|
Term
| effects of alpha thalassemia |
|
Definition
| alpha chains are a component of all types of hemoglobin, so alpha thalassemia affects alpha and beta chains. |
|
|
Term
| pts with transfusion dependent thalassemia can develop what? |
|
Definition
| iron orverload that leads to CHF - these pts are often treated with desferrixoamine (chelating agent) |
|
|
Term
| how do you diagnose beta-thalassemia |
|
Definition
hemoglobin electrophoresis - increased hemoblogin F
peripheral blood smear - microcytic hypochromic anemia |
|
|
Term
| tx of beta thalassemia major (Cooley's anemia). |
|
Definition
| frequent PRBC transfusion |
|
|
Term
| dx of b-thalassemia minor |
|
Definition
| heterozygous b-chain thalassemia |
|
|
Term
| types of alpha thalassemia |
|
Definition
silent carriers - mutation/deletion of only one a locus
a-thalassemia trait - mutation/deletion of two alpha loci
Hg H disease - mutation at 3 alpha loci
tx = frequent PRBC transfusion, splenectomy can be helpful
mutation at all 4 loci is either fatal at or shortly after birth |
|
|
Term
| clinical findings of siderblastic anemia |
|
Definition
increased serum iron and ferritin
normal TIBC
ringed sideroblasts in bone marrow |
|
|
Term
| causes of anemia of chronic disease (4) |
|
Definition
chronic infection - TB, lung abscess
cancer - lung, breast, Hodgkin's
inflammation - RA, SLE
trauma
release of cytokines has a suppressive effect on erythropoeisis |
|
|
Term
low serum iron
low TIBC
low serum transferrin levels
increased serum ferritin levels |
|
Definition
| anemia of chronic disease |
|
|
Term
| causes of aplastic anemia (5) |
|
Definition
idiopathic (majority)
radiation exposure
meds - chloramphenicol, sulfonamides, gold, carbamazepine
viral infection - parvovirus, hep C, Hep B, EBV, CMV, zoster, HIV
chemicals - benzene, insectacides |
|
|
Term
|
Definition
bone marrow transplantation
transfusion of PRBC and platelets (use judiciously)
immunosuppression |
|
|
Term
| how mcuh B12 do we store in the body? |
|
Definition
| B12 stores in the liver can last a person for 3 or more years |
|
|
Term
| main dietary sources of B12 |
|
Definition
|
|
Term
| how/where is B12 absorbed? |
|
Definition
| bound to intrinsic factor (produced by gastric parietal cells),so it can be absorbed by the terminal ileum |
|
|
Term
| MCC of B12 deficiency anemia |
|
Definition
|
|
Term
| how does pernicious anemia differ from B12 deficiency anemia |
|
Definition
| pernicious anemia is an autoimmune disorder resulting in inadequate production of intrinsic factor and thus impaired absorption of B12 |
|
|
Term
| lab findings in b12 deficiency anemia |
|
Definition
megaloblastic anemia
hypersegmented neutorphils
B12 levels < 100 pg/mL
serum methylmalonic acid and homocysteine levels are elevated |
|
|
Term
| diagnostic test for B12 deficiency |
|
Definition
|
|
Term
| how much folic acid does the body store? |
|
Definition
| limited - inadequate intake over 3 months can lead to deficiency |
|
|
Term
|
Definition
| green vegetables - overcooking them can remove the folate |
|
|
Term
|
Definition
|
|
Term
|
Definition
| suggest intravascular hemolysis ("trauma" or mechanical hemolysis) |
|
|
Term
| spherocytes of helmet cells suggest |
|
Definition
|
|
Term
| pathogenesis of sickle cell diesase |
|
Definition
| hemoglobin S replaces hemoglobin A |
|
|
Term
| aplastic crisis in sickle cell anemia can be precipitated by |
|
Definition
viral infections such as human parvovirus B19.
tx = blood transfusion |
|
|
Term
| clinical findngs in sickle cell anemia secondary to vaso-occlusion |
|
Definition
painful crisis involving the bone
hand-foot syndrome - painful swelling of dorsa ofhands and feet seen in infancy and childhood caused by AVN of the metacarpal and metatarsal bones.
acute chest syndrome - due to repeated episodes of pulmonary infarctions
repeated splenic infarctions
AVN in joints - MC = hip
priapism
CVA
ophthalmologic complications
chronic leg ulcers
abdominal crisis |
|
|
Term
| splenic sequestration syndrome |
|
Definition
| sudden pooling of blood into the spleen results in rapid development of splenomegaly and hypovolemic shock - potentially fatal complication of sickle cell disease and B-thalassemia. MC in children because they have intact spleens. |
|
|
Term
| sickle cell patients should be vaccinated against what? |
|
Definition
| pneumoniae, H influenzae, N. meningitidis |
|
|
Term
| prophylactic tx of kids with sickle cell disease |
|
Definition
|
|
Term
|
Definition
hydration
pain mangement with morpine
keep pt warm
supplemental O2
hydroxyurea - enhances Hb F |
|
|
Term
| lab findings in hereditary spherocytosis |
|
Definition
RBC osmotic fragility
elevated retic count and elevated MCHC
blood smear = sphere shaped RBCs
direct coombs test is negative |
|
|
Term
| tx of choice for hereditary spherocytosis |
|
Definition
|
|
Term
| known precipitants of G6PD deficiency |
|
Definition
| sulfonamides, nitrofurantoin, primaquine(antimalarial), dimercaprol, fava beans, infection |
|
|
Term
| clinical features of G6PD |
|
Definition
more severe in people of Mediterranean descent
episodic hemolytic anemia that is usually drug induced
dark urne and jaundice |
|
|
Term
|
Definition
blood smear = bite cells and heinz bodies
deficient NADPH formation on G6PD assay
low levels of G6PD (althogh may be normal during hemolytic episode) |
|
|
Term
| autoimmune hemolytic anemia (AIHA) |
|
Definition
| production of autoantibodies toward RBC membrane antigen which leads to destruction of these RBCs |
|
|
Term
|
Definition
warm - IgG binds optimally at warm temps causes extravascular hemolysis. causes = lymphoma, leukemia, collagen vascular disease, drugs
cold - IgM binds well at cold temps causes intravascular hemolysis. causes = idiopathic or |
|
|
Term
|
Definition
warm - IgG binds optimally at warm temps causes extravascular hemolysis. causes = lymphoma, leukemia, collagen vascular disease, drugs
cold - IgM binds well at cold temps causes intravascular hemolysis. causes = idiopathic or infection. |
|
|
Term
|
Definition
direct coombs test
RBC's coated with IgG = positive = warm AIHA
RBC's coated with complement alone - dx is cold AIHA |
|
|
Term
|
Definition
often none is necessary.
warm - glucocorticoids, splenectomy, immunosuppression, RBC transfusion, folic acid supplement.
cold - avoid exposure to cold, RBC transfusion, chemotherapeutic agents, steroids are not beneficial! |
|
|
Term
| definition of thrombocytopenia |
|
Definition
|
|
Term
| types of heparin induced thrombocytopenia |
|
Definition
HIT1 - heparin directly causes platelet aggregation, seen < 48 hrs after initiating
HIT2 - heparin induced antibody mediated injury to platelet - seen 3-12 days after initating heparin. heparin should be d/c immediately |
|
|
Term
| clinical features of thrombocytopenia |
|
Definition
cutaneous bleeding
mucosal bleeding
excessive bleeding after procedures or surgery
intracranial hemorrhage and heavy GI bleeding |
|
|
Term
|
Definition
seen in kids and preceded by a viral infection.
usually self limited - 80% resolve spontaneously |
|
|
Term
|
Definition
| usually seen in adult women age 20-40, spontaneous remissions are rare. |
|
|
Term
|
Definition
platelet count usually < 20,000
bone marrow aspiration = increased megakaryocytes
increased amount of platelet associated IgG |
|
|
Term
|
Definition
adrenal corticosteroids
IV immune globulin
splenectomy
platelet transfusions |
|
|
Term
| hwo does TTP affect PT and PTT? |
|
Definition
| unaffected because there is no consumption of clotting factors |
|
|
Term
|
Definition
plasmapheresis (large volume)
corticosteroids and splenectomy
platelet transfusions are contraindicated! |
|
|
Term
|
Definition
| fever, altered mental status, HUS (microangiopathic hemolytic anemia, thrombocytopenia, renal failure) |
|
|
Term
|
Definition
| fever, altered mental status, HUS (microangiopathic hemolytic anemia, thrombocytopenia, r |
|
|
Term
| abnormally large platelets on peripheral smear think... |
|
Definition
|
|
Term
| MC inherited bleeding disorder |
|
Definition
|
|
Term
| 3 subtypes of vWF disease |
|
Definition
1 = decreased
2 = abnormal
3 = absent |
|
|
Term
| lab findings with vWF disease |
|
Definition
| prolonged bleeding time - normal PT, normal or prolonged PTT |
|
|
Term
| tx of von willebrands disease |
|
Definition
DDAVP - desmopressin (not effective in type 3)
factor 8 concentrates
avoid aspirin, NSAIDs, and cryoprecipitate |
|
|
Term
| characteristic type of bleeding for hemophilia |
|
Definition
| hemarthrosis, intracranial bleeding, intramuscular hematomas, retroperitoneal hematomas, hematuria or hemospermia |
|
|
Term
| lab findings in hemophilia A |
|
Definition
|
|
Term
|
Definition
acute hemarthrosis - codeine with or without acetaminophen - avoid ASA/NSAID
clotting factor replacement - factor 8 concentrate
DDAVP |
|
|
Term
|
Definition
| limited to factor 9 concentrates |
|
|
Term
| what do PT, PTT, thrombin time, and bleednig time reflect? |
|
Definition
PT = extrinsic pathway (warfarin)
PTT = intrinsic pathway (heparin)
TT = fibrinogen concentration
BT = platelet function |
|
|
Term
| normal PT, PTT, and bleeding time |
|
Definition
PT = 11-15 seconds
PTT = 25-40 seconds
BT = 2-7 minutes |
|
|
Term
|
Definition
infectin
obstetric complications
major tissue injury
malignancy
shock
snake venom (rattlesnakes) |
|
|
Term
| differentiate vitamin K deficiency and liver disease |
|
Definition
liver disease - PT and PTT are elevated. TT, fibrinogen, and platelets are usually normal
vitamin K deficiency - Pt is prolonged, PTT, TT, platelet count, fibrinogen level S are normal |
|
|
Term
|
Definition
increased PT, PTT, BT, TT, fibrin split products, D-dimer
decreased - fibrinogen level, platelet count
peripheral smear reveals schistocytes |
|
|
Term
| vitamin K dependent clotting factors |
|
Definition
| 2, 7, 9, 10, protein C and S |
|
|
Term
| pts with antithrombin 3 deficinecy don't respond to what medication? |
|
Definition
|
|
Term
| protein c deficiency leads to |
|
Definition
| increase in facotrs 5 and 8, which leads to unregulated fibrin synthesis |
|
|
Term
| factor 5 leiden deficinecy leads to |
|
Definition
| unregulated prothrombin activation and thus an increase in thrombotic events |
|
|
Term
| inherited hypercoagulable states (7) |
|
Definition
antithrombin 3 deficiency
antiphospholipid antibody syndrome
Protein C deficiency
Protein S deficiency
Factor 5 Leiden
prothrombin gene mutation
hyperhomocystinemia |
|
|
Term
|
Definition
PTT by inhibiting factors 2a and 10a.
half life of standard heparin is 1 hr
half life of LMWH is 3-24 hrs |
|
|
Term
| contraindications to heparin |
|
Definition
previous HIT
active bleeding, GI/intracranial bleed
hemophilia, thrombocytopenia
severe HTN
recent surgery on eyes, spine, or brain |
|
|
Term
|
Definition
half life is short, will cease to have effect 4 hrs after discontinuation
reverse effects with protamine sulfate.
administer FFP if severe bleeding occurs. |
|
|
Term
|
Definition
| PT by inhibiting vitamin K dependent clotting factors |
|
|
Term
| administration of warfarin |
|
Definition
start heparin first - once PTT is therapeutic being warfarin
continue heparin for atleast 4 days, once INR is therapeutic stop heparin and continue warfarin.
goal INR = 2-3 |
|
|
Term
| reversing the effects of warfarin |
|
Definition
vitamin K - can correct abnormal PT wihtin 4-10 hours. can make it difficult to return to the pt to therapeutic INR levels.
if significant bleed give FFP |
|
|
Term
|
Definition
low hemoglobin
high calcium
high serum protein
poor renal function
lytic lesions in the low back, chest (ribs), or jaw. |
|
|
Term
|
Definition
|
|
Term
|
Definition
serum and urine protein electrophoresis - monoclonal spike of IgG
atleast 10% abnormal plasma cells
lytic bone lesions
hypercalcemia
high serum protein
peripheral smear - rouleaux formation
elevated ESR
urine - bence jones proteins
leukopenia, thrombocytopenia, anemia |
|
|
Term
|
Definition
systemic chemo
radiation therapy if no response to chemo and if disabling pain is present
transplantation - autologous peripheral blood stem cell transplantation |
|
|
Term
| how is Hodgkin's disease staged? |
|
Definition
Ann Arbor staging system -
1 = single lymph node
2 = 2+ lymph nodes on same side of diaphragm
3 = both sides of diaphragm
4 = extralymphatic dissemination |
|
|
Term
| suffixes for Hodkin's disease |
|
Definition
A = no constitutional sxs
B = weight loss, night sweats, - presence of constitutional sxs worsens prognosis |
|
|
Term
| bimodal distribution of Hodgkin Disease |
|
Definition
|
|
Term
| clinical featurse of Hodgkin Lymphoma |
|
Definition
painless LAD
supraclavicular, cervical, axillary, mediastinal lymph nodes
spreads continuously from one lymph node to an adjacent lymph node |
|
|
Term
|
Definition
Reed Sternberg cells (look like owl eyes)
presence of inflammatory cell infiltrates (distinguishes from NHL)
CXR and CT to detect lymph node involvement
boen marrow biopsy
labs - leukocytosis, eosinophilia, ESR |
|
|
Term
|
Definition
| chemo and radiation therapy |
|
|
Term
|
Definition
HIV/AIDS
immunosuppression
hx of viral infection
hx of H Pylori gastritis
autoimmune disease |
|
|
Term
|
Definition
lymph node biopsy >1cm for > 4wks
CXR - hilar or mediastinal adenopathy |
|
|
Term
| tx of intermediate and high grade NHL |
|
Definition
CHOP therapy
Cyclophosphamide
Hydroxydaunomycin (doxorubicin)
Oncovin (vincristine)
prednisone |
|
|
Term
| MC malignancy in children under age 15 |
|
Definition
|
|
Term
| myelogenous leukemia affects what cells |
|
Definition
| granulocytes or monocytes |
|
|
Term
| leukemia with testicular involvement |
|
Definition
|
|
Term
| leukemia with skin nodules |
|
Definition
|
|
Term
| auer rods are pathognomonic for |
|
Definition
|
|
Term
|
Definition
|
|
Term
| smude cells are pathognomonic for |
|
Definition
|
|
Term
|
Definition
potential complication of chemotherapy seen in acute leukemia and high grade NHL.
rapid cell death with release of intracellular contects caueses hyperkalemia, hyperphosphatemia, and hyperuricemia.
treat as a medical emergency |
|
|
Term
|
Definition
|
|
Term
| diagnostic criteria for polycythemia vera |
|
Definition
must have all 3 major plus any 2 minor
Major: elevated RBC mass, O2 sat >92%, splenomegaly
Minor: thrombocytosis (>400), leukocytosis (>12), leukocyte alk phos (>100), serum B12 (>900) |
|
|
Term
|
Definition
repeated phlebotomy
myelosupprsesion with hyroxyurea or recombinant interferon alfa |
|
|
Term
| secondary causes of polycythemia vera (2) |
|
Definition
hypoxemia
carbon monoxide exposure |
|
|
Term
| dx of myelodysplastic syndromes |
|
Definition
bone marrow biopsy - blasts or ringed sideroblasts
Howell Jolly Bodies |
|
|
Term
| tx of mylodysplastic syndromes |
|
Definition
mainly supportive
RBC and platelet transfusions
erythropoeitin
granulocyte colony stimulating factor
vitamin supplementation, B6, B12 and folate
Pharm therapies - immunosuppression, cehmo, androgenic steroids
bone marrow transplant = only potential cure |
|
|
Term
| definition of essential thrombocytosis |
|
Definition
|
|
Term
| lab findings in thrombocytosis |
|
Definition
splenomegaly, pseudohyperkalemia, elevated bleedng time
bone marrow biopsy - megakaryocytes
smear = hypogranular, abnormally shaped platelets |
|
|
Term
|
Definition
antiplatelet agents - anagrelide and low dose aspirin
hydroxyurea is sometimes used if severe |
|
|
Term
| hallmark feature of agnogenic myeloid metaplasia with myelofibrosis |
|
Definition
| teardrop cells on peripheral smear |
|
|
