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HemeReview 2013
Clinical Medicine
| Question | Answer |
|---|---|
| IDA (causes) | -chronic blood loss --> GI tract most common -Chronic NSAID/ASA use -Pregnancy -menstrual blood loss -low iron intake (women, children) |
| IDA (ssx) | -pallor, fatigue, DOE, tachycardia -*pica* -esophageal webs (plummer-vinson syndrome) |
| IDA (dx) | -hypochromic, microcytic anemia -elevated TIBC -low Fe, ferritin, and transferritin |
| IDA (tx) | -tx underlying cause -iron supplementation with ferrous sulfate x 6-12 months |
| Lead Poisoning (ssx) | -difficulty concentrating, fatigue, muscle weakness, paralytic ileus |
| Lead Poisoning (dx) | -mild microcytic anemia -basophilic stippling of RBC -elevated lead lvl |
| Lead Poisoning (tx) | -remove lead source -chelating agents if symptomatic or severe toxicity |
| Thalassemia | -inherited disorder of alpha or beta globulin synthesis -typically family hx -genetic counseling in sever forms |
| Thalassemia (dx) | -microcytic anemai that does not respond to iron therapy -normal iron and ferritin levels -confirm w/ hbg electrophoresis |
| alpha thal (dx) | -peripheral smear: target cells -Hgb H on electrophoresis |
| alpha thal (tx) | -Avoid iron -avoid oxidative drugs -folic acid supplements |
| beta thal major | -Cooley anemia -onset 4-6 months of age, seveer anemia, growth retardation, osteopenia, deformities, hepatosplenomegaly |
| beta thal minor | -moderate anemia |
| beta thal (dx) | -peripheral smear: basophilic stippling, target cells -microcytic |
| beta thal major (tx) | -transfusion with deferoxamine to prevent hemosiderosis |
| beta thal minor (tx) | -avoid iron -monitor |
| B12 deficiency | -vegans, pancreatic insufficiency, gastric bypass surgery, crohn's |
| B12 deficiency (exam) | -glossitis, pale icterus -neuro: stocking-glove paresthesias, loss of tast and vibratory sensation, ataxia |
| B12 deficiency (Dx) | -macrocytic anemia, poikilocytosis, anisocytosis -low retic count and serum B12 -high LDH and bilirubin |
| B12 deficiency (tx) | -high dose oral B12, nasal spray or IM |
| B12 deficiency (neuro ssx) | -will resolve if tx is initiated within 6 months of onset -if not corrected damage is irreversible |
| pernicious anemia | -autoimmune destruction of gastric parietal cells causing lack of intrinsic factor -most common cause of B12 deficiency |
| pernicious anemia (dx) | -macrocytic anemia -low serum B12 -shilling test positive |
| pernicious anemai (tx) | -lifelong IM B12 supplement |
| folic acid deficiency | -poor intake, EtOH abuse, pregnancy, bactrim, suflasalazine |
| folic acid deficiency (dx) | -macrocytic -hypersegmented PMNCs -folate level <150 ng/mL |
| folic acid deficiency (tx) | -folic acid 1 mg/day by mouth |
| hemolytic anemias (cuases) | -TTP, ITP, DIC -medication/transfusion reactions -sickle cell -G6PD deficiency -ifxns (malaria, parvo b19) |
| hemolytic anemias (exam) | -splenomegaly |
| hemolytic anemias (dx) | -elevated retic; falling hct -elevated indirect bilirubin -elevated LDH |
| sickle cell anemia | -autosomal recessive hemolytic anemia; african americans |
| sickle cell anemia (aggravators) | -sickling of RBCs increased by hypoxemia, high altitudes |
| sickle cell anemia (prognosis) | -life expectancy = 40-50 yrs |
| sickle cell anemia (ssx) | -painful crises, cholelithiasis, poor wound healing, priaprism, splenomegaly, AVN of femoral head |
| sickle cell anemia (dx) | -Hgb electrophoresis shows Hgb S in RBCs -sickled cells, target cells, howell-jolly bodies |
| sickle cell anemia (tx: symptomatic) | -analgesics, fluids, O2 |
| sickle cell anemia (tx: crises) | -possible transfusion |
| sickle cell anemia (tx: asx) | -pneumococcal vaccine/folate supplements -genetic counseling |
| G6PD deficiency | -X linked recessive; mediterranean populations and african american males |
| G6PD deficiency (path) | -leads to episodic hemolysis or chonic hemolysis if severe |
| G6PD deficiency (risks) | -oxidative drugs; infxn -fava beans |
| G6PD deficiency (dx) | -bite cells, heinz bodies -G6PD low after hemolytic episode |
| G6PD deficiency (tx) | -most cases are self-limited -avoid oxidative meds (ASA, dapsone, sulfonamides, nitrofurantoin) |
| ACD (anemia of chronic dz) | -associated w/ kidney, liver, endocrine dz, or malignancy -renal dz leads to decreased EPO |
| ACD (dx) | -mild normochromic, normocytic anemia -normal cell morphology |
| ACD (tx) | -treat underlying dz and anemia will resolve -give EPO |
| aplastic anemia (ssx) | -weakness, bleeding, bacterial and fungal infections, purpura, petichiae, hepatosplenomagaly |
| aplastic anemia (causes) | -most common: T cell mediated autoimmune suppression -radiation, chemotherapy, medications, SLE |
| aplastic anemia (dx) | -pancytopenia w/ normocytic anemia -hypocellular marrow |
| aplastic anemia (tx: mild) | -supportive; EPO/transfusions |
| aplastic anemai (tx: severe) | -bone marrow transplant |
| polycythemia vera (primary) | -JAK 2 mutation -splenomegaly, normal O2 sat, increased red cell mass |
| polycythemia vera (secondary) | -chronic hypoxia, smoking, renal tumors -splenomegaly absent |
| polycythemia vera (who?) | -males>females -age>60 |
| polycythemia vera (complications) | -bleeding (PUD/epistaxis), thrombosis, progression to leukemia |
| polycythemia vera (ssx) | -tinnitus, HA, dizziness, blurred vision, plethora -pruritis after bathing |
| polycythemia vera (dx) | -elevated Hct -leukocytosis, increased large/bizarre platelets -hypercellular bone marrow |
| polycythemia vera (tx) | -phlebotomy -hydroxyurea -low dose ASA |
| thrombocytopenia | -decrease in the number of platelets -most common cause of abnormal bleeding -associated w/ SLE and CLL |
| thrombocytopenia (ssx) | -gingival bleeding, epistaxis, menorrhagia, petichiae |
| thrombocytopenia (causal drugs) | -heparin, sulfonamides, thiazides, cimetidine |
| ITP | -autoimmune, IgG mediated |
| acute ITP (ssx) | -petechia, purpura, hemorrhagic bullae on skin |
| acute ITP (tx) | -most are self-limiting -steroids -splenectomy if severe |
| acute ITP (who?) | -children, following a viral infection |
| chronic ITP (who?) | -women>men; ages 20-50 yrs |
| chronic ITP (ssx) | -petechiae on skin and mucous membranes -associated w/ autoimmine conditions (SLE) |
| chronic ITP (tx) | -high dose steroids; IVIG; SCT -splenectomy -avoid ASA -platelet transfusion for severe bleeding |
| TTP (who?) | -women>men; age 20-50; previously healthy |
| TTP | -associated w/ HIV, pregnancy, estrogen, quinine, ticlopidine -rare but can be fatal |
| TTP (ssx) | -severe thrombocytopenia, anemia (schistocytes), elevated LDH, thrombosis -pallor, petechiae, purpura, pancreatitis, fever -neurologic signs that come and go |
| TTP (tx) | -emergent plasmapheresis (NO PLATELETS) -prednisone -antiplatelet agents |
| DIC | -SAS -occurs pts w/ sever systemic illnesses; sepsis, cancer, transfution reactions, trauma -high mortality (30-80%) |
| DIC (ssx) | -shock, hemorrhage from skin and mucous membranes, digital ischemia, gangrene |
| DIC (labs) | -coagulopathy: elevated D-dimer, prolonged PT |
| DIC (tx) | -urgently treat underlying cause -component blood transfusions -cyroprecipitate -+/- heparin |
| vW dz | -most common congenital coagulopathy -deficiency of facter VIII antigen and defective vWF |
| vW dz (gentics) | -autosomal dominant bleeding disorder |
| vW dz (ssx) | -bleeding of nasal passages, vagina, GI tract |
| vW dz (labs) | -prolonged bleeding time -decreased vWF |
| vW dz (tx) | -desmopressin acetate -factor VIII concentrates -avoid ASA and exogenous estrogen -pregnancy may exacerbate symptoms |
| hemophilia A | -most common hemophilia; factor VIII deficiancy -most severe bleeding disorder: 2nd most common congenital coagulopathy |
| hemophilia (genetics) | -x-linked recessive |
| hemophilia A (ssx) | -range from severe hemarthrosis, intracranial bleeding to milder prolonged post op bleeding |
| hemophilia A (labs) | -prolonged PTT -low factor VIII:C levels -normal vWF |
| hemophilia A (tx) | -heat treated factor VIII concentrates -mild dz can be treated w/ desopressin -avoid ASA |
| hemophilia B | -factor IX deficiency or Christmas dz -x-linked recessive; males |
| hemophilia B (tx) | -factor IX concentrates -FFP (fresh frozen plasma) |
| Vit K deficiency | -most common acquired coagulopathy |
| Vit K deficiency (causes) | -chronic liver dz (most common), dietary, meds |
| Vit K deficiency (ssx) | -post surgical pts; soft tissue bleeding |
| Vit K deficiency (labs) | -PT/PTT are prolonged -elevated LFTs -decreased Factors VII, IX, X, II (SNOT) |
| Vit K deficiency (tx) | -oral or IV vit K -treat acute bleeds with FFP |
| thrombotic disorders | -pts: >40, family history, recurrent thrombosis |
| aquired thrombotic disorders | -malignancy, estrogen use, immobilization, nephrotic syndrome, ulcerativ colitis, crohn's, heparin |
| congenital thrombotic disorders | -antithrombin III deficiency, factor V leiden, protein C&S deficiencies, lupus anticoagulant |
| thrombotic disorders (tx) | -anticoagulation w/ LMWH or coumadin -predinsone for lupus anticoagulant |
| hodgkin's dz (who?) | -men>women; ages 15-45 |
| hodgkin's dz | -enlargement of lymphoid tissue, liver/spleen -associated w/ EBV |
| hodgkin's dz (ssx) | -painless LAD, fever, night sweats, pruritis, fatigue -pain w/ EtOH |
| hodgkin's dz (dx) | -ann arbor creteria -staging -staging tests: CT neck, chest, abdomen, pelvis, BM bx -*reed-sternberg cells* |
| hodgkin's dz (tx) | -combinations chemotherapy or radiation depending of stage at diagnosis -majority of pts are cured |
| non-hodgkin's lymphoma | -malignancy of lymphocytes (MC B lymphocytes) -age 20-40; more common with HIV/immune compromise |
| non-hodgkin's lymphoma (ssx) | -painless persistent LAD -abdominal fullness in Burkitt's lymphoma -spread to bone, bone marrow, GI, skin |
| non-hodgkin's lymphoma (labs) | -biopsy enlarged nodes -stage w/ CXR, CT abdomen and pelvis -BM bx |
| non-hodgkin's lymphoma (tx) | -radiation for single node -rituximab +/- chemotherapy -high grade: sct -spontaneous remission may occur |
| multiple myeloma | -malignancy of the plasma cells -more common in AA |
| multiple myeloma (ssx) | -osteolytic lesions, LAD, recurrent infections |
| multiple myeloma (labs) | -hypercalcemia -*rouleaux formation* of RBCs -monoclonal spike on electrophoresis (M spike on SPEP) -bence-jones proteins in urine |
| multiple myeloma (tx) | -dexamethason, doxorubicin, lenalidomide -bisphosphonates for osteoporosis |
| acute leukemia (ssx) | -epistaxis, gingival bleeding, gram-negatic bacterial infxns -anemai, DIC, elevated uric acid |
| acute leukemia (dx) | -pancytopenia w/ circulating blasts -elevated WBCs -confirm w/ BM bx |
| acute leukemia (tx) | -induction chemotherapy followed by consolidation therapy -allopurinol/diuretcs to prevent uric acid stones -sct |
| ALL | -most common childhood leukemia |
| ALL (ssx) | -rapid onset of fever, fatigue, joint pain, bone pain (sternum, femor), frank bleeding |
| ALL (cxr) | -mediastinal mass |
| ALL (labs) | -leukocytosis w/ lymphocytosis -philadelphia chromosome (poor prognosis) |
| ALL (tx) | - >50% cure rate for children undergoing chemotheraphy |
| AML (who?) | -adults: median onset of age 60 -MC acute leukemia |
| AML (ssx) | -gradual onset of fatigue, decreased appetite, weight loss, and dyspnea |
| AML (dx) | -*auer rods* |
| AML (tx) | -high remission rate in pts < 60 |
| CLL (who?) | -MC of all leukemias -men>women -median age of onset 65 years |
| CLL | -malignancy of B lymphocytes, median survival 6 years |
| CLL (ssx) | -recurrent infections, LAD, Richter's syndrome |
| CLL (staging) | -Rai system |
| CLL (dx) | -lymphocytosis -peripheral smear: *smudge cells* |
| CLL (tx) | -palliative w/ advanced dz |
| CML | -myeloproliferative disorder seeen in middle aged adults |
| CML (ssx) | -gradual onset of weight loss, low grade fever, fatigue, sweating, splenomegaly, early satiety |
| CML (triphasic) | -progression from chronic to accelerated to acute phase (blast crisis/decreased survival |
| CML (dx) | -leukocytosis -*philadelphia chromosome* -bcr-abl gene identification -hypercellular bone marrow |
| CML (tx) | -imantinib mesylate(Gleevec), dasatinib, nilotinib -allogenic sct only curativ therapy (reserved for progressive disease) |
Created by:
duanea00
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