MKSAP18 Flashcards
What type of shock could result from a patient with methemoglobinemia?
Dissociative Shock (tissue hypoxia); pt with distributive shock or high levels of methemoglobin (>4) should be tx with IV methylene blue
Patients with localized GIST at increased risk of recurrence s/p resection should be tx with 3 years of ________ after surgery; what form of conventional cancer therapy are GIST resistant to
Imatinib (although rare, GIST is the MC sarcoma of the GI tract); of note, GIST is resistant to radiation
What is the most appropriate tx for primary HIV associated thrombocytopenia in pt with newly diagnosed HIV without bleeding?
HARRT, recall that ITP is a diagnosis of exclusion–PHAT (Primary HIV associated thrombocytopenia) is the diagnosis here; steroids do have efficacy in PHAT but they increase immunosuppression so are not preferred
What is more common in adults–hepatic sequestration or splenic sequestration?
Hepatic sequestration; this is a reaction in sickle cell patients and splenic sequestration is more rare bc most adults have infarcted the spleen
A pt with SOB, hypoxia, and hypotension IMMEDIATELY after start of blood product is likely having what type of transfusion reaction?
Anaphylactic; TRALI would not be IMMEDIATE but an hour or so; TACO would not have hypotension but rather hypertension and volume overload
The findings of a large tongue (macroglossia) with lateral scalloping is indicative of this disease
AL amyloidosis
What are three cancers associated with elevated levels of EPO production?
HCC, RCC, and Pheochromocytoma
What is the most appropriate blood product for a pt with ESLD and hypofibrinogenemia who is bleeding (i.e. hematemesis)
Cryoprecipitate (technically 1 unit/10 kg) but most just do 10 unit; At levels less than 100, pts at increased risk of bleeding; NOTE: cryo comes from FFP but the volume of FFP to give in order to get the fibrinogen up would be excessive
Cancer associated hypercoagulability can lead to this hypercoaguble syndrome characterized by migratory superficial thrombophlebitis
Trosseau syndrome (also similar underpinning to nonbacterial thrombotic endocarditis–marantic endocarditis) and is related to chronic DIC d/t release of tissue factor from cancer cells
What infusion rate of blood can decrease the chances of further episodes of TACO?
Infuse slowly at 1 mL/kg/hr rather than the usual 2-3 mL/kg/hr
What is the likely etiology of severe thrombocytopenia developing within 24 hours of MI with catheterization?
Administration of a GpIIb/IIIa inhibitor i.e. Abciximab (ReoPro) or Eptifibitide; preformed antibodies against neoepitopes on GpIIbIIIa are exposed by binding of drug
Most likely dx with pt with absolute leukocytosis >100k, basophilia, thrombocytosis and splenomegaly
CML with t(9;22) BCR-ABL; CMML is negative BCR-ABL but often with SRSF2 and TET2 mutations and >10% persistent monocytosis
A patient with frequent and serious bacterial and fungal infections with a negative nitroblue tetrazolium test has _______
Chronic Granulomatous Disease; Tx = Bacterial ppx with TMP-SMX and Itraconazole for Fungus as well as IFN-Y for immunomodulatory agent
What are the usual immunophenotypic markers of CLL?
CD5+ and CD23+; CD10 not so much that is more follicular; CD10 would be unusual for MCL too; CD23 in MCL usually neg
Women with a personal of FMHx of ovarian CA, endometrial CA, and colon CA should undergo testing for ________
Lynch Syndrome (AD susceptibility syndrome w/ mutations in MLH1, MSH2, and MSH6 which are MMR (mismatch repair) genes; the somatic “second hit” causes CA)
What is the likely diagnosis for a pt with sickle cell disease with RUQ, tender hepatomegaly with severe anemia and reticulocytosis and distributive shock?
Hepatic sequestration
In the absence of hairy villous projections, a suspected diagnosis of HCL can be made how?
They often have CD103 positivity, high levels of soluble CD25 (Soluble IL2R) and TRAP positivity; cladribine first line
How would you know that the malignant cells in mycosis fungoides were clonal?
They are CD4 positive T cells and would have a dominant TCR gene rearrangement
What are the effects of calcium and vitamin C on iron absorption?
Inhibited by calcium (also levothyroxine and tetracyclines affected); iron absorption increased by vitamin C
What is a high homocysteine level very sensitive for?
Pretty sensitive but not specific for folic acid deficiency; it is high w/ normal MMA and B12 in folate def and high with high MMA in B12 def
What may be a differentiating historical factor to consider when diagnosing PNH vs. Aplastic Anemia with some PNH clones present?
PNH more often presents with thrombotic issues and not just hypocellular marrow; however, both may have some hemolysis (more in PNH), hypocellularity, and hypoplastic MDS may also be on ddx but again, thrombosis not as common in those
What is the deal with breast CA screening in women who have had mantle radiation for Hodgkin lymphoma?
Should be getting annual breast MRIs and Mammograms (I think Diagnostic)
What is the treatment of many low grade NETs and/or hormonally functional NETs
Octreotide or lanreotide; of note, platinum based therapy (cisplatin and etoposide) is more for high grade NETs including SCLC
How would an acute eosinophilic leukemia present?
Likely with a leukemic presentation with immature eosinophils and BM infiltration with cytopenias; can have cardiac dysfunction like in HES and ABPA
What may be the cause of pancytopenia WITHOUT myeloid immaturity or leukoerythroblastosis but with bone pain, massive splenomegaly and no LAD?
Gaucher Disease (tissue paper macrophage); AR disorder of glucocerebrosidase enzyme with accumulation of glycolipids
The test used to confirm a diagnosis of autoimmune hemolytic anemia is _____; smear can suggest this by findings of _______
DAT (direct Coombs); marked anemia and microspherocytes
Which form of NHL often has presence in the GI tract even if not evident on imaging?
Mantle Cell Lymphoma t(11;14) overexpress CCND1 = Cyclin D1; study found that blind bx of normal GI mucosa often showed it; there is also the syndrome of Lymphomatous Polyposis
What are drepanocytes? What are echinocytes?
Drepanocytes are the technical term for sickled cells; Echinocytes are seen in uremia and liver dz they are burr cells and have smaller and more uniform spicules rather than acanthocytes
What is suggested by the combination of mild anemia with infrequent pain crises in a patient with splenomegaly and occasional drepanocytes?
Hemoglobin SC disease; unique because often SS has autoinfarction of spleen and not splenomegaly (but I have seen SC pts with asplenia)
What is the MCV often in aplastic anemia?
Usually macrocytic but with BM cellularity of <25% with ANC <500 or platelet <20 or absolute retic count <20 k
What is functional iron deficiency in CKD?
Transferrin saturation <30% or ferritin <500 could suggest that they still need iron
What did the TRICC trial (1999) show?
Restrictive strategy of transfusion to hgb of 7 was at least as good as liberal (10-12) with a trend towards higher mortality in ischemic heart dz but not statistically significant
Based on results from intensive ALL trials in pediatrics, this drug is often included in AYA regimens (under 40) but excluded from adults
Asparaginase (Pegasparaginase); CALGB 10403 is the regimen
This is diagnosed several days after a transfusion with a profound decrease in the hemoglobin level with reticulocytosis and concomittant increase in T bili and LDH
Delayed Hyperhemolytic reaction due to amnestic response of preformed alloantibody after reexposure to antigen; the fact that there is a RETICULOCYTOSIS helps differentiate from aplastic crisis
What is the initial therapy of mycosis fungoides?
Topical steroids but if fails can do retinoids (bexarotene), psoralen and ultraviolet light (PUVA) etc; patients with more extensive or infiltrative diseases can get CHOP (general tx of peripheral T cell lymphomas), HDACs (romidepsin, vorinostat) or alemtuzumab (anti-CD52)
What therapy should be considered for young patients with advanced stage CLL associated with a high risk of disease progression such as del 17p
Would need some sort of remission induction but ultimately allo-HSCT if young and good PS (for 17p, predicts for lack of benefit to standard chemo i.e. FCR so might do ibrutinib or idelalisib)
What is the appropriate workup of a patient with iron deficiency and thrombocytosis who has a persistent thrombocytosis after corrected the iron?
Evaluation for Essential Thrombocythemia–i.e. MC cause is a reactive thrombocytosis so correcting the iron def should improve the plt count but if not check JAK2V617F; recall though, that JAK2 neg in 50% of ET
These are inclusion bodies in RBCs that are seen in patients who are functionally or anatomically asplenic
Howell-Jolly bodies (so can be seen in Sickle Cell dz as well)
What is generally added to patients with stage II-III NSCLC s/p successful resection (Regardless of histologic type i.e. squamous, adeno, adenosquamous)?
Adjuvant platinum based chemo (I think usually cisplatin/paclitaxel)
Gray platelet syndrome is named as such because __________
Absence of alpha granules make platelet look gray on EM; this is an inherited qualitative platelet defect
What should you think of in a person with prolonged and reversible coagulopathy (i.e. responds to FFP and vitamin K) but then gets worse again frequently?
Superwarfarin Poisoning (Rat poison) managed with FFP and several months of tapering vitamin K until the agent is eliminated (high INR with deficiencies in II, VII, IX, and X)
The most likely diagnosis for chronic asymptomatic neutropenia in a black person is ________ and is related to polymorphisms in this gene _______
Benign Ethnic Neutropenia; DARC (Duffy Antigen for Chemokines)
Aside from Mantle Cell lymphoma, what other disorder commonly has a t(11;14)?
Multiple Myeloma can often have this with overexpression of CCND1 gene which encodes cyclin D1
What is the most likely diagnosis for a pt with long standing HIV and isolated thrombocytopenia? Tx and how is it different if newly dx HIV?
If long standing would think ITP and tx is steroids; if new dx prob HIV assoc and can do HARRT first
What do the direct antiglobulin and indirect antiglobulin tests test for?
DAT is to test for antibodies to red cells i.e. for AIHA (either warm or cold) and indirect is the screen of a type and screen and tests for antibodies
What is the goal ferritin in patients with hereditary hemochromatosis? What is the treatment if the patients have anemia due to phlebotomy?
Under 50; continue phlebotomy through the anemia; iron chelation is not really done in hereditary hemochromatosis but is done in other secondary forms
A patient with vWF disease who bleeds after a surgery despite prophylaxis with desmopressin should receive _________
Von Willebrand Factor Concentrate i.e. Humate P, Alphanate, Wilate, or Koate HP
A Heinz body can be seen if this stain is used when evaluating for G6PD?
Crystal Violet
What is the major treatment for TMA of pregnancy or HELLP?
Delivery of the baby not much else available to Tx
Where is EPO made?
Interstitial peritubular fibroblasts of kidney; routine measurement of EPO in anemia of CKD is not needed
What are the main 4 types of microcytic anemia?
Iron deficiency, inflammatory, sideroblastic anemia, and thalassemia
Why would the LDH be elevated in patients with B12 deficiency?
There is intramedullary hemolysis in B12 deficiency due to ineffective hematopoiesis leading to RBC turnover can also be seen in bone marrow failure syndromes such as MDS, aplastic anemia, and inherited disorders (i.e. Diamond Blackfan etc)
What are the 3 possible translocations of C-myc in Burkitt lymphoma?
t(8;14) is MC and classic translocation of C-myc to IgH; t(2;8) translocation involving kappa chain; t(8;22) translocation involving lambda chain
The finding of bite cells and Heinz bodies on a smear of pt with hemolytic anemia after drug exposure is pathognomonic for ________
G6PD deficiency
What coagulation issue are patients with beta-thalassemia intermedia at increased risk of?
Thrombosis–leads to abnormal erythrocytes that express procoagulants and phospholipids; of note: beta thalassemia minor is asymptomatic; occasional need for transfusion is beta thalassemia intermedia, and profound anemia often needing transplant is beta thal major
What is the main preventative strategy to decrease future episodes of TACO?
Slow the infusion rate to 1 mL/kg/hr rather than 3 and give lasix
What can be said of Factor XII deficiency?
Do NOT exhibit bleeding despite an in vitro high PTT; factor XIII deficiency on the other hand (fibrin stabilizing factor def) can have normal PT and PTT but can have bleeding
Reversal agents for dabigatran? Factor Xa inhibitors?
Idaracizumab, andexanet alfa
What are 3 important risk factors for progression of MGUS to MM?
Presence of a non-IgG M-protein; M-spike >1.5 (Recall >3 = MM), Abnormal serum free light chain ratio
This is the best initial therapy for patients with suspected TTP
PLEX
This should be offered to all patients with SCLC who have a complete or near-complete response to chemotherapy or concurrent chemoradiation
Prophylactic WBRT to decrease chances for brain mets
What should patients with hereditary hemochromatosis avoid in terms of food?
Avoid raw or undercooked seafood as Vibrio vulnificus in raw oysters is high risk (additionally, excess iron appears to impair host defenses against certain infxn and decreases chemotactic response)
