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USMLE2 Medicine 10
Hematology
| Question | Answer |
|---|---|
| bone marrow filled with plasma cells, serum with functionless Ig's | multiple myeloma |
| most common initial sx of multiple myeloma | bone pain in back and ribs (pathologic fractures), radiculopathy from nerve root compression, hyperCa |
| multiple myeloma is xxchromic and xxcytic anemia | normochromic, normocytic |
| Protein electrophoresis with markedly elevated monoclonal immunoglobulin spike | multiple myeloma |
| xray with punched out lytic lesions | overproduction of osteoclast activating factor from plasma cells, multiple myeloma |
| elevated B2 microglobulin | multiple myeloma |
| Bence Jones protein | multiple myeloma, found via acidification of Bence Jones protein |
| dx multiple myeloma | BM bx with >10% plasma cells |
| Tx of multiple myeloma in younger pts | thalidomide and prednisone --> autologous BMT (stem cells from self) |
| Tx of multiple myeloma in older pts | melphalan, prednisone, and thalidomide. PMT |
| tx of hyperCa | loop diuretics (furosemide) and then bisphosphonate (pamidronate) |
| MGUS | asx overproduction of Ig by plasma cells |
| who gets MGUS | >50 |
| Tx of MGUS | not necessary |
| Reed Sternberg cells | Hodgkin lymphoma |
| who gets Hodgkin lymphoma? | bimodal - 20's and 60's |
| pt with enlarged, painless, rubbery nonerythematous, nontender cervical/supravclavicular/axillary LN's | Hodgkin lymphoma |
| B sx's | night sweats, 10% wt loss, fever |
| Hodgkin/non-Hodgkin lymphoma Stage I | one lymphatic grp or one extra-lymphatic site |
| Hodgkin/non-Hodgkin lymphoma Stage II | two lymphatic grps or extra-lymphatic site on same side of diaphragm |
| Hodgkin/non-Hodgkin lymphoma Stage III | lymphatic grps on both sides of diaphragm OR any extra-lymphatic organ contig to primary nodal site |
| Hodgkin/non-Hodgkin lymphoma Stage IV | widespread disease with diffuse extralymphatic sites such as BM or liver |
| dx of Hodgkin/non-Hodgkin lymphoma | excisional LN bx |
| adverse prognostic factors in Hodgkin lymphoma | LEBA (LAD, ESR, B sx, Age): Large mediastinal LAD, >40yo, B sx's, inc ESR |
| Tx for Hodgkin/non-Hodgkin lymphoma | Stages I and II: radiation, B sx's or stage III and IV: for Hodg, ABVD (adriamycin [doxorubicin], bleomycin, vinblastine, dacarbazine); for non-Hodgkin CHOP (cyclophosphamide, Hydroxy-adriamycin, oncovin (vincristine), prednisone) |
| which Hodgkin lymphoma with best prognosis? which has worst prognosis? | lymphocyte predominant is best; lymphocyte depleted is worst |
| which lymphoma associated more with extralymphatic spread? | non-Hodgkin lymphoma |
| What infections predispose to development of non-Hodgkin lymphoma? | HIV, Hep C, EBV, HTLV-1 and H.pylori |
| HIV and EBV most often associated with.... | Burkitt lymphoma |
| enlarged painless rubbery nonrythematous nontender LN's, PLUS extra lymphatic / blood involvement | non-Hodgkin lymphoma |
| relapse of non-Hodgkin lymphoma tx with what? | autologous BMT |
| which non-Hodgkin lymphoma pt should get rituximab? | those with lots of CD20 cells (rituximab is anti-CD20, inc survival when added to CHOP) |
| Idiopathic Thrombocytopenic Purpura (ITP) | Ab to plt --> eaten by macrophage --> removal by spleen --> dec plt count |
| ITP associated with what? | CHCL (chuckle): Lymphoma, CLL, HIV, and connective tissue diseases |
| bone marrow in ITP filled with what? | megakaryocytes (prob is plt destruction, not production because plenty of megakaryocytes) |
| Tx for ITP | 1) prednisone, 2) 10K to 20K plt - splenectomy, 3) <10K plt IVIG or Rhogam in (Rh+ pts since binds to Rh antigen on RBCs, only pts WITH spleens), 4) rituximab as last resort |
| plt type bleeding | epistaxis, petechiae, bruising, menstrual abnl |
| inheritance of vWD | autosomal dominant |
| what is the problem in vWD? | decreased ability of plt to adhere to endothelial lining of blood vessels (plt aggregation still nl) |
| plt count and appearance in vWD | both are normal |
| test to dx vWD | ristocetin platelet aggregation (tests plt ability to bind to an artificial endothelial surface -ristocetin) |
| PT/PTT in vWD | PT nl, PTT elevated (decrease in factor 8) |
| Tx for vWD | 1. DDAVP (release vWF), 2. Factor 8 (contains vWF). |
| What med shouldn't person with vWD NOT use? | ASA |
| Diseases with prolonged PT | extrinsic pathway (on R). Factor 7 deficiency/inhibitor, Vit K def, Liver dz, warfarin. |
| diseases with prolonged PTT | intrinsic pathway (on L). vWF/Factor 8 9 11 12 def, heparin, APL Ab |
| Diseases with prolonge PT and PTT | Def/inhib: prothrombin, fibrinogen, factor 5, factor 10. DIC. Supratherapeutic heparin or warfarin. |
| Hemophilia A | def of factor 8 |
| Hemophilia B | def of factor 9 |
| hemophilia inheritance pattern | X-linked recessive --> males only because females who are homozygous have intrauterine death |
| which is more common hemophilia - A or B? | A (factor 8) |
| factor-type bleeding (as opposed to plt type bleeding) | deeper: hemarthroses, hematoma, GI bleed, urinary bleed, bruising, CNS bleeding |
| PT/PTT profile of hemophilia | prolonged PTT (intrinsic) and nl PT (extrinsic) |
| mixing study. what does it mean if PTT normalizes? what does it mean if PTT doesn't normalize? | hemophilia - 50:50 mix of pt blood with normal blood brings PTT to nl. if does not normalize, then think of inhibitors of Factors 8 or 9 |
| tx for mild hemophilia | Hemophilia A: DDAVP, also use before surgery, or replacement of Factor 8. Hemophilia B: FFP, cryotherapy. |
| What factors affected with Vit K def? | 2 7 9 10 |
| In vit K def, what is affected? PT PTT | both. PT first and more severe. |
| Tx of Vit K def | severe - infusion of FFP. Also give Vit K at the same time (takes a while to kick in) |
| what clotting factors not made in liver? | Factor 8 and vWF |
| which coag factor depleted first in liver disease | Factor 7 |
| most common site of bleeding in liver disease | GI tract |
| how to tx severe bleeding in liver disease | FFP acutely. long term - tx liver disease. |
| DIC | pathologic activation of coagulation with consumption of plt and clotting factors. small blood clots form inside vessels --> consume coagulation proteins and platelets --> normal coagulation is disrupted and abnormal bleeding occurs |
| microangiopathic hemolysis ass with | DIC |
| d-dimers associated with.... What are they? | ass with DIC. a fibrin degradation product, present after a blood clot is degraded by fibrinolysis |
| fibrinogen levels in pt with DIC, d-dimers | fibrinogen low because it's being consumed. d-dimer high because it's a fibrin degradation prod released after blood clot is degraded, which is happening a lot in DIC |
| Tx DIC | FFP and plt transfusions, correct underlying disorder |
| if supratherapeutic INR >9, <20, how to tx | hold warfarin and give 5 to 10 oral VitK. resume warf at lower dose once INR is therapeutic |
| if supratherapeutic INR >20, how to tx | hold warfarin and give 10 IV VitK, FFP. resume warf at lower dose once INR is therapeutic |
Created by:
christinapham
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