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78 Cards in this Set
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anemia generalities
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presentation --> fatigue, tiredness, exercise intolerance, dyspnea, tachycardia, angina, palpitations, lightheadedness, confusion
Hct < 41% in men or 36% in women hemoglobin <13.5mg/dL in men or 12mg/dL in women MCV < 80 --> microcytic; MCV >100 --> macrocytic |
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anemia general etiology
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microcytic --> iron deficiency, thalassemia, sideroblastosis, lead poisoning
macrocytic --> folate or B12 deficiency, alcohol, liver disease, methotrexate, AZT, phenytoin normocytic --> sickle cell, paroxysmal nocturnal hemoglobinuria, hereditary spherocytosis, G6PDH deficiency, drug-induced AOCD can be micro or normocytic |
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anemia general diagnosis
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determine hematocrit and hemoglobin
if anemia --> MCV then iron levels, reticulocyte count, peripheral smear, RDW, Coombs, B12/folate levels or marrow biopsy depending on on the case |
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anemia general treatment
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packed red blood cells to maintain hematocrit > 25-30%
hematocrit rises 3 points for every unit of red blood cells |
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iron deficiency anemia
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general anemia symptoms plus brittle spoon-shaped nails, glossitis, pica
ferrtin<10ng/mL is highly specific but low sensitivity ↓serum iron, ↑TIBC, ↓MCV, ↓reticulocytes ferrous sulfate tablets or parenteral iron in some cases |
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anemia of chronic disease
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can't use storage iron of reticuloendothelial system with consequent excess
due to neoplasia, systemic inflammation or infection general symptoms of anemia normal or ↑ferritin, ↓serum iron and TIBC treat underlying disease iron or erythropoietin will not help except in renal failure or chemotherapy |
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sideroblastic anemia
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defect in Hb synthesis with trapped iron in mitochondria of RBCs
genetic --> ALA deficiency or defect in B6 metabolism acquired --> chloramphenicol, isoniazid, alcohol, lead poisoning can be associated with myelodysplastic syndromes and can progress to AML initial tests --> ↑ferritin, ↑serum iron, ↓TIBC confirmation --> marrow smear shows ringed sideroblasts in Prussian blue stain treatment --> remove causing agent, lead and iron chelators, B6 in some cases |
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alpha thalassemia presentation
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1 gene deletion is asymptomiaic
2-gene deletion presents with mild anemia (Hct 30-40%) and profound microcytosis 3-gene deletion presents with severe anemia (Hct 20-30%) and profound microcytosis 4-gene deletion patients die in utero |
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alpha thalassemia diagnosis and treatment
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anemia with profound microcytosis and target cells are clues
normal iron studies with microcytosis make diagnosis Hb electrophoresis determines type and has normal HbF and HbA2 with HbH in 3 or 4 gene deletion no specific treatment required |
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beta thalassemia presentation
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beta thalassemia minor --> mild anemia and profound microcytosis
beta thalassemia major --> profound anemia and microcytosis, symptoms start when HbF decreases 6 months after birth; growth failure, bone deformities, hepatosplenomegaly, jaundice beta thalassemia major requires monthly transfusions which lead to iron overload (managed with deferasirox), hemochromatosis, cirrhosis, CHF |
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beta thalassemia diagnosis and treatment
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anemia + microcytosis
electrophoresis shows increased HbA2 and HbF beta thalassemia major requires monthly transfusions and deferasirox for iron overload may do splenectomy or marrow transplant |
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B12 deficiency etiology
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atrophic gastritis, gastrectomy, Crohn, sprue, pancreatic insuficiency, D. latum
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B12 deficiency presentation
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anemia of varying severity and neurologic symptoms of any kind alone or in combination
may have glositis, diarrhea or abdominal pain |
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B12 deficiency diagnosis and treatment
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made by showing anemia and macrocytosis
anti-intrinsic factor antibodies in pernicious anemia hypersegmented neutrophils, macro-ovalocytes hypercellular marrow, pancytopenia ↑LDH, ↑billirubin, ↑iron may do Schilling test treat --> intramuscular B12 |
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Folic acid deficiency
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due to ↓intake (diet, alcoholics)
↑requirements (pregnancy) ↑loss (eczema, dialysis, phenytoin) general anemia presentation depending on severity diagnosis --> ↓Hb and ↓folic acid levels may show macro-ovalocytes, hypersegmented neutrophils |
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sickle cell disease etiology
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autosomal recessive substitution of valine for glutamic acid at sixth amino acid of beta globin chain; painful crisis can be spontaneous or precipitated by fever, acidosis, dehydration, infection and fever
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sickle cell disease presentation
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symptoms of anemia
chronic manifestations --> isothenuria, ulcerations of the skin of the legs, bilirubin gallstones, asceptic necrosis of femoral head, osteomyelitis, retinopathy, recurrent pneumococcus or Haemophilus infections, growth retardation, splenomegaly/splenectomy acute painful crisis --> back, rib, chest and leg pain acute chest syndrome --> indistiguishable from pneumonia, severe chest pain, fever leukocytosis, hypoxia, x-ray lung infiltrates, pripapism, blindness, MI, abortions |
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sickle cell disease diagnosis
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mild to moderate anemia plus normal MCV
sickle cells on peripheral smear high reticulocyte count ↑bilrubin, ↑LDH microhematuria leukocytosis electrophoresis is the most specific test also sickle cell prep test |
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sickle cell disease management
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acute pain crisis --> fluids, analgesics, oxygen
if fever/leukocytosis --> ceftriaxone or cefotaxime severe manifestations such as priapism and cardiac --> packed RBCs if hematocrit is low or exchange transfusion if high chronic management --> folic acid, pneumococcus and influenza vaccinations and hydroxyurea to decrease frequency of vaso-oclusive/pain crisis |
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autoimmune/drug-induced hemolytic anemia etiology
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IgG antibodies against RBC membrane with destruction mostly in spleen by macrophages
idiopathic or secondary to leukemias (CLL), lymphoma, viral infections collagen vascular diseases, lupus penicillins, cephs, sulfas, quinidine, methyldopa, procainamide, rifampin, thiazides |
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autoimmune/drug-induced hemolytic anemia presentation
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symptoms related to severity
sudden onset may have fever, syncope, CHF, hemoglobinuria, weakness, pallor, jaundice, dark urine |
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autoimmune/drug-induced hemolytic anemia diagnosis and treatment
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normocytic anemia, reticulocytosis, ↑LDH, ↓haptoglobin, ↑indirect bilirubin
Coombs test is specific mild disease does not require specific treatment steroids in more severe disease splenectomy if unresponsive |
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cold agglutinin anemia etiology
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IgM against RBC membrane in response to lymphoma, Waldenstrom, mycoplasma or mononucleosis, ulcerative colitis
RBCs destroyed in liver |
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cold agglutinin anemia presentation
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symptoms related to severity
cyanosis in nose, ears, fingers and toes weakness, pallor jaundice, dark urine |
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cold agglutinin anemia diagnosis and treatment
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normocytic anemia, reticulocytosis, ↑LDH, ↓haptoglobin, ↑indirect bilirubin, often spherocytes
Coombs test is specific mild disease does not require specific treatment steroids or splenectomy won't work managed by avoiding cold weather or with azathiorpine, cyclosporine, cyclophosphamide, rituximab (anti-CD20 Ab) |
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hereditary spherocytosis etiology
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autosomal dominant defect in spectrin of RBC membrane;
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hereditary spherocytosis presentation
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mild to moderate symptoms of anemia, spherocytes
splenomegaly and jaundice sometimes cholecystitis |
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hereditary spherocytosis diagnosis and treatment
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anemia, normal MCV, ↑LDH, ↑reticulocytes, ↑indirect bilirubin
spherocytes and negative Coombs test positive osmotic fragility test treat with folate or splenectomy |
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paroxysmal nocturnal hemoglobinuria etiology
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red cell membrane defect in phosphatidyl inositol glycan A leads to increased complement lysis and intravascular hemolysis
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paroxysmal nocturnal hemoglobinuria presentation
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symptoms of anemia acording to severity
hemoglobinuria (specially in morning urine) thrombosis (specially hepatic vein, Budd-Chiari) |
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paroxysmal nocturnal hemoglobinuria diagnosis and treatment
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↓DAF, hematuria
normal MCV, ↑LDH, ↑reticulocytes, ↑indirect bilirubin, ↓haptoglobin specific tests are sugar-water test and acidified hemolysis test treat with folate, corticosteroids and anticoagulation for thrombosis |
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G6PDH deficiency
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enzyme deficiency leads to ↓NADPH, ↓gluthathione peroxidase activity and ↑unmetabolized H2O2 which precipitates Hb (Heinz bodies)
precitpitated by infections, sulfa drugs, primiquine, quinidine, dapsone, nitrofurantoin presentation --> sudden-onset anemia symptoms, jaundice, dark urine normal MCV, ↑indirect bilirubin, ↑LDH, ↑reticulocytes, ↓haptoglobin, hemoglobinuria, Heinz bodies, bite cells definitive test is ↓G6PDH treat with hydrration |
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aplastic anemia etiology
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most common is idiopathic; radiation, benzene, NSAIDs, chloranphenicol, alcohol, alkylating agents; infections: hepatitis, HIV, CMV, EBV, ParvoB19;
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aplastic anemia presentation
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bleeding from thrombocytopenia
fatigue, anemia and may have infections from neutropenia may have history of using precipitating drugs |
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aplastic anemia diagnosis and treatment
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screening --> pancytopenia
confirm --> bone marrow biopsy showing absence of marrow cells, fat and no signs of cancer, infection or fibrosis in marrow treat --> bone marrow transplant; if not, antithymocyte globulin, cyclosporine and prednisone |
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acute leukemia etiology
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most have no apparent cause; associated with radiation, benzene, melphalan, etoposide, retroviruses, Down and Klinefelter, myelodysplasia and sideroblastic anemia
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acute leukemia presentation
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fatigue, anemia and bleeding from thrombocytopenia +- leukopenia or leukocytosis
ALL --> in children, hepatosplenomegaly, lymphadenopathy, bone pain AML --> DIC (promyelocytic M3), CNS meningitis-like (monocytic M4, M5) |
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acute leukemia diagnosis
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pancytopenia although leukocytes can be up, down or normal
leukemic blasts in peripheral blood bone marrow biopsy showing >20% blasts confirms diagnosis to differentiate --> AML --> Auer rods (specially M3), myeloperoxidase and esterase ALL --> CALLA and TdT specific types of leukemia are diagnosed with monoclonal antibodies |
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acute leukemia treatment
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AML --> AraC with danourubicin or idarubicin
ALL --> daunorubicin, vincristine and prednisone add all-trans-retinoic acid in promyelocytic leukemia remission is removal of 99.9% of leukemic cells and elimination of blasts in peripheral blood |
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differential diagnosis for pancytopenia
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marrow --> aplastic anemia, metastatic cancer to marrow, myelofibrosis
infectious --> parvirus, retrovirus auto-immune --> B12 deficiency, SLE, hypersplenism drugs --> chloramphenicol acute leukemia is only one that shows blasts in peripheral blood |
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chronic myelogenous leukemia etiology
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clonal disorder of myelocytes associated in 95%with translocation t(9,22) (Philadelphia chromosome) which produces an enzyme with tyrosine kinase activity
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chronic myelogenous leukemia presentation
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leukocytosis and sometimes thrombocytosis lead to
fatigue, night sweats, low-grade fever abdominal pain, splenomegaly, bone pain rare --> lymphadenopathy, infection or bleeding leukostasis has dyspnea, blurry vision, priapism, thrombosis, stroke |
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chronic myelogenous leukemia diagnosis
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leukocytosis (mostly neutrophils)
↓LAP (difference with leukemoid reaction) absent or very low blast count if leukocytosis and ↓LAP --> check for Philadelphia chromosome |
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chronic myelogenous leukemia treatment
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imatinib is an inhibitor of the tyrosine kinase produced by disease
90% hematologic response and 60-70% loose Philadephia chromosome if imatinib doesn’t work --> bone marrow transplant |
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chronic lymphocytic leukemia presentation
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older patients can be asymptomatic
0 --> lymphocytosis alone 1 --> lymphadenopathy 2 --> splenomegaly 3 --> anemia 4 --> thrombocytopenia infection and bleeding are unusual survival of stage 0-1 is 10-12 years survival of 3-4 is 1-2 years |
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chronic lymphocytic leukemia diagnosis
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suspected in older patient with leukocytosis (80-98% lymphocytes)
bone marrow biopsy confirms leukemic lymphocytes smudge cells on peripheral smear |
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chronic lymphocytic leukemia treatment
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stages 0-1, no specific treatment
symptomatic patients are given fludarabine (DOC) or chlorambucil prednisone for autoimmune hemolysis and thrombocytopenia pentostatin for relapses |
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multiple myeloma presentation
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bone pain (specially back, ribs), lytic lessions, osteoporoosis, pathologic fractures
hypercalcemia (polyuria, polydipsia, altered mental status) renal failure (Bence Jones light chains) infection with encapsulated bugs anemia |
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multiple myeloma diagnosis
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normo normo anemia, hypercalcemia, ↑BUN, ↑creatinine
lytic bone lessions on x-rays Bence Jones protein ↑serum microglobulin B2 (75%) electrophoresis with monoclonal IgG spikes cofirmation --> bone marrow biopsy showing >10%plasma cells confirms |
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multiple myeloma treatment
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younger patients --> transplant (pre-treat with thalidomide, dexamethasone)
older patients --> melphalon, prednisone, thalidomide treat hypercalcemia with loop diuretics |
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monoclonal gammopathy of uncertain origin
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overproduction of clonal immunoglobulin by plasma cells without systemic manifestations
patient is asymptomatic clue to diagnosis is elevated serum protein monoclonal Ig spike on protein electrophoresis no bone lessions, no anemia, no renal failure, no hypercalcemia no treatment necessary |
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Hodgkin disease presentation
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nontender lynmphadenopathy
cervical, supraclavicular or axillary nodes are most common B symptoms are drenching night sweats, 10% weight loss, fever |
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Hodgkin disease diagnosis
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first step --> excisional lymph node biopsy showing Reed-Sternberg cells
second step --> determine staging with CT if CT is unrevealing and radiotherapy is contemplated then do laparotomy also anemia, leukocytosis, eosinophillia, ↑LDH, ↑ESR (adverse prognosis) may be seen |
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Hodgkin disease adverse prognostic factors
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large mediastinal lymphadenopathy
age older than 40 B symptoms ↑ESR |
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Hodgkin disease treatment
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stage I and II --> radiotherapy
B symptoms or stage III or IV --> ABVD chemo (adriamycin, bleomycin, vinblastine, dacarbazine) has less side effects than MOPP chemo (meclorethamine, oncovin/vincristine, prednisone, procarbazine) potential side effects --> permanent sterility, cancer, leukemia, aplastic anemia, peripheral neuropathy |
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non-Hodgkin lymphoma etiology
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HIV, hepatitis C, EPV, HTLV-1, H. pylori
HIV and EPV are associated with Burkitt and high-grade high-stage lymphomas |
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non-Hodgkin lymphoma presentation
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nontender lymphadenopathy with rubbery consistency
B symptoms --> drenching night sweats, 10% weight loss, fever cervical/supraclavicular --> 10-20% more likley to involve extra-lymphatic sites |
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non-Hodgkin lymphoma diagnosis
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first test --> excisional lymph node biopsy which excludes Reed-Sternberg cells
staging --> CT; laparotomy is not needed may do marrow biopsy also anemia, leukocytosis, eosinophillia, ↑LDH, ↑ESR may be seen |
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non-Hodgkin lymphoma treatment
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stages I and II --> radiotherapy
B symptoms or stages II or III --> CHOP chemo (cyclophosphamide, hydroxy-adriamycin, oncovin/vincristine, prednisone) if CNS lymphoma give radiotherapy with CHOP treat relapse with bone marrow transplant |
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Hodgkin and non-Hodgkin staging
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stage I --> one lymphatic group or extra-lymphatic tissue
stage II --> two lymphatic groups or extra-lymphatic tissue on same side of diaphragm stage III --> lymph nodes on both side of diaphragm or extralymphatic involvement contiguous to primary site stage IV --> widespread lymph involvement or diffuse extra lymphatic sites |
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idiopathic thrombocytopenic purpura
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antibodies against platelets leads to destruction and bleeding
epistaxis, bruising, petechiae, echymoses hematuria, dysfunctional uterine bleeding, GI bleeding thrombocytopenia is found and splenomegaly is absent exclude HUS, TTP, DIC --> peripheral smear without schistocytes and normal creatinine exclude marrow thrombocytopenia --> marrow biopsy with normal megakaryocytes treat with prednisone or splenectomy if unresponsive |
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von Willebrand disease etiology
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autosomal dominant defect in factor VIII production results in inability of platelets to adhere to vascular endothelium; aggregation is normal
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von Willedrand disease presentation
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petechiae, echymoses, epistaxis, bruising
hematuria, dysfunctional uterine bleeding, GI bleeding increased with aspirin |
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von Willebrand disease diagnosis
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platelet count is normal, ↑PTT, ↑BT
↓factor VIII antigen (vWB factor) abormal ristocetin test |
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von Willebrand disease treatment
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desmopressin used in mild bleeding and before minor surgery
if moderate to severe symptoms give factor VIII avoid aspirin |
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causes of prolonged PT only
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inherited --> factor VII deficiency
acquired --> vitamin K deficiency, liver disease, warfarin |
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causes of prolonged PTT only
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inherited --> vWF, factors VIII, IX, XI or XII deficiencies
acquired --> heparin, antiphospholipid antibody |
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causes of prolonged PT and PTT
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inherited --> prothrombin, fibrinogen, factors V or X deficiencies
acquired --> liver disease, vitamin K deficiency, DIC, supratherapeutic heparin or warfarin, inhibitors (thrombin, prothrombin, fibrinogen, factor V or factor X) |
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hemophilia etiology
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x-linked recessive; manifests in males; females are carriers
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hemophilia presentation
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obvious by age 2, apparent at time of circumcision
mild deficiencies --> asymptomatic severe deficiencies (<5-10% activity) --> factor-type bleeding, hemarthrosis, hematoma, GI or urinary bleeding, bruising or CNS bleeding |
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hemophilia diagnosis
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prolonged PTT, normal PT
mixing study indicates deficiency when PTT corrects to normal in 50:50 mixture patient and control blood if it doesn't correct, factor inhibitor is cause, not deficiency specific factor VIII or IX levels are necessary for precise diagnosis |
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hemophilia treatment
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mild hemophilia A is treated with desmopressin
severe deficiencies treated with specific factor replacement desmopressin doesn't work in hemophilia B |
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vitamin K deficiency
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decreased factors 2, 7, 9, 10 due to dietary deficiency of vitamin K, malabsorption or antibiotics
presentation --> hemarthrosis, hematoma, GI or urinary bleeding evident as oozing at venapuncture sites PT and PTT are elevated diagnosis --> confirmed after vitamin K injection and PT/PTT correction treat severe bleeding with fresh frozen plasma and vitamin K infusion |
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liver disease coagulopathy
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decreased clotting factors except 8 and vWB
due to severe liver disease or cirrhosis presents with bleeding, elevation of PT and PTT clinically indistinguishable from vitamin K deficiency except that it doesn’t correct with infusion low platelets due to acompanying hypersplenism treat with fresh frozen plasma and management of liver condition |
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disseminated intravascular coagulation etiology
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idiopathic
sepsis pancreatitis promyelocytic leukemia rhabdomyoloysis adenocarcinomas heatstroke transfusion reactions burns trauma abruptio placenta amniotic fluid embolism snakebites |
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disseminated intravascular coagulation presentation
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factor-type and platelet-type bleeding at any site
hemolysis can lead to acute renal failure, jaundice, confusion |
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disseminated intravascular coagulation diagnosis
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↑PT/PTT
↓platelets ↓fibrinogen ↑fibrinogen split products and d-dimers schistocytes present in peripheral smear |
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disseminated intravascular coagulation treatment
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fresh frozen plasma and maybe platelet transfusions; correct underlying disorder
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