MKSAP18 Flashcards
1
Q
What type of shock could result from a patient with methemoglobinemia?
A
Dissociative Shock (tissue hypoxia); pt with distributive shock or high levels of methemoglobin (>4) should be tx with IV methylene blue
2
Q
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
A
Imatinib (although rare, GIST is the MC sarcoma of the GI tract); of note, GIST is resistant to radiation
3
Q
What is the most appropriate tx for primary HIV associated thrombocytopenia in pt with newly diagnosed HIV without bleeding?
A
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
4
Q
What is more common in adults–hepatic sequestration or splenic sequestration?
A
Hepatic sequestration; this is a reaction in sickle cell patients and splenic sequestration is more rare bc most adults have infarcted the spleen
5
Q
A pt with SOB, hypoxia, and hypotension IMMEDIATELY after start of blood product is likely having what type of transfusion reaction?
A
Anaphylactic; TRALI would not be IMMEDIATE but an hour or so; TACO would not have hypotension but rather hypertension and volume overload
6
Q
The findings of a large tongue (macroglossia) with lateral scalloping is indicative of this disease
A
AL amyloidosis
7
Q
What are three cancers associated with elevated levels of EPO production?
A
HCC, RCC, and Pheochromocytoma
8
Q
What is the most appropriate blood product for a pt with ESLD and hypofibrinogenemia who is bleeding (i.e. hematemesis)
A
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
9
Q
Cancer associated hypercoagulability can lead to this hypercoaguble syndrome characterized by migratory superficial thrombophlebitis
A
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
10
Q
What infusion rate of blood can decrease the chances of further episodes of TACO?
A
Infuse slowly at 1 mL/kg/hr rather than the usual 2-3 mL/kg/hr
11
Q
What is the likely etiology of severe thrombocytopenia developing within 24 hours of MI with catheterization?
A
Administration of a GpIIb/IIIa inhibitor i.e. Abciximab (ReoPro) or Eptifibitide; preformed antibodies against neoepitopes on GpIIbIIIa are exposed by binding of drug
12
Q
Most likely dx with pt with absolute leukocytosis >100k, basophilia, thrombocytosis and splenomegaly
A
CML with t(9;22) BCR-ABL; CMML is negative BCR-ABL but often with SRSF2 and TET2 mutations and >10% persistent monocytosis
13
Q
A patient with frequent and serious bacterial and fungal infections with a negative nitroblue tetrazolium test has _______
A
Chronic Granulomatous Disease; Tx = Bacterial ppx with TMP-SMX and Itraconazole for Fungus as well as IFN-Y for immunomodulatory agent
14
Q
What are the usual immunophenotypic markers of CLL?
A
CD5+ and CD23+; CD10 not so much that is more follicular; CD10 would be unusual for MCL too; CD23 in MCL usually neg
15
Q
Women with a personal of FMHx of ovarian CA, endometrial CA, and colon CA should undergo testing for ________
A
Lynch Syndrome (AD susceptibility syndrome w/ mutations in MLH1, MSH2, and MSH6 which are MMR (mismatch repair) genes; the somatic “second hit” causes CA)
16
Q
What is the likely diagnosis for a pt with sickle cell disease with RUQ, tender hepatomegaly with severe anemia and reticulocytosis and distributive shock?
A
Hepatic sequestration
17
Q
In the absence of hairy villous projections, a suspected diagnosis of HCL can be made how?
A
They often have CD103 positivity, high levels of soluble CD25 (Soluble IL2R) and TRAP positivity; cladribine first line
18
Q
How would you know that the malignant cells in mycosis fungoides were clonal?
A
They are CD4 positive T cells and would have a dominant TCR gene rearrangement
19
Q
What are the effects of calcium and vitamin C on iron absorption?
A
Inhibited by calcium (also levothyroxine and tetracyclines affected); iron absorption increased by vitamin C
20
Q
What is a high homocysteine level very sensitive for?
A
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
21
Q
What may be a differentiating historical factor to consider when diagnosing PNH vs. Aplastic Anemia with some PNH clones present?
A
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
22
Q
What is the deal with breast CA screening in women who have had mantle radiation for Hodgkin lymphoma?
A
Should be getting annual breast MRIs and Mammograms (I think Diagnostic)
23
Q
What is the treatment of many low grade NETs and/or hormonally functional NETs
A
Octreotide or lanreotide; of note, platinum based therapy (cisplatin and etoposide) is more for high grade NETs including SCLC
24
Q
How would an acute eosinophilic leukemia present?
A
Likely with a leukemic presentation with immature eosinophils and BM infiltration with cytopenias; can have cardiac dysfunction like in HES and ABPA
25
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
26
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
27
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
28
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
29
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)
30
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
31
What is functional iron deficiency in CKD?
Transferrin saturation \<30% or ferritin \<500 could suggest that they still need iron
32
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
33
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
34
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
35
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)
36
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)
37
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
38
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)
39
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)
40
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
41
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)
42
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)
43
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
44
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
45
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
46
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
47
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
48
A Heinz body can be seen if this stain is used when evaluating for G6PD?
Crystal Violet
49
What is the major treatment for TMA of pregnancy or HELLP?
Delivery of the baby not much else available to Tx
50
Where is EPO made?
Interstitial peritubular fibroblasts of kidney; routine measurement of EPO in anemia of CKD is not needed
51
What are the main 4 types of microcytic anemia?
Iron deficiency, inflammatory, sideroblastic anemia, and thalassemia
52
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)
53
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
54
The finding of bite cells and Heinz bodies on a smear of pt with hemolytic anemia after drug exposure is pathognomonic for \_\_\_\_\_\_\_\_
G6PD deficiency
55
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
56
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
57
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
58
Reversal agents for dabigatran? Factor Xa inhibitors?
Idaracizumab, andexanet alfa
59
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
60
This is the best initial therapy for patients with suspected TTP
PLEX
61
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
62
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)
63
For patients with GE junction cancer, the _______ trial showed superiority of pre-op and post-op chemo was better than surgery alone. What was the chemo?
MAGIC; Epirubicin + Cisplatin + 5-FU; I think now just Cisplatin and 5-FU and don?t forget to check HER2 status for trastuzumab
64
This is the best therapy for patients with ET who are over 60 years of age or who have a platelet count over one million or if they have prior hx of thrombosis?
ASA and Hydroxyurea (of note, anegrelide is inferior to hydrea and hydrea can decrease thrombosis risk EVEN if platelet count not decrease; Ruxolitinib only if painful spleen or sx from MF)
65
This is the best therapy for patients with uremia and bleeding
DDAVP as it increases plasma levels of vWF and FVIII; however, dialysis is another option for bad uremic bleeding
66
What is a possible diagnosis for a patient with HCV who develops splenomegaly with diffuse LAD?
Splenic Marginal Zone Lymphoma (can regress with TX of the HCV)
67
Which patients with essential thrombocythemia should receive both aspirin and hydroxyurea?
Those who are \>60 or who have history of thrombosis (and some will say if at risk of thrombosis)
68
Pancytopenia associated with splenomegaly, B sx and monocytopenia with atypical lymphocytes is very consistent with \_\_\_\_\_\_\_\_\_\_
Hairy Cell Leukemia (a leukemia that often presents with cytopenias); the cytopenias are due to marrow infiltration by hairy cells and by splenic sequestration d/t splenomegaly; often CD103 positive and high levels of soluble CD25
69
Patients with an INR \<9 and no evidence of bleeding who are warfarin can be tx how?
Holding one or two doses of warfarin and in this situations the CHEST guidelines recommend against giving vitamin K; if greater than 9 can give some vitamin K but if bleeding it is never sufficient to give vitamin K usually need PCC
70
What is the function in vivo of ADAMTS-13? What is the utility in diagnosing TTP?
It cleaves ultra-large von willebrand multimers so when left uncleaved they build up and can cause MAHA; not necessary for dx but if \<10% it is rather characteristic
71
The CALGB 10403 regimen is most likely to be used in ALL patients in what setting?
AYA (Adolescent and Young Adult) i.e. under 40; otherwise more of a Hyper-CVAD regimen more so than an asparaginase containing one
72
What sorts of disorders need to be considered in patients with leukoerythroblastosis?
MPNs particularly PMF or disorders with secondary fibrosis but also granulomatous disease, metastatic tumor, thalassemia
73
How is the bleeding pattern of acquired hemophilia different from inherited hemophilia?
Acquired hemophilias tend to be both mucocutaneous and deep rather just deep bleeds; don?t forget to work up WHY they have an inhibitor i.e. SPEP/UPEP etc.
74
What should you think in a person with microcytic anemia, target cells on smear and normal RDW?
Thalassemia either alpha or beta; if mild it is either alpha minor or beta minor and can only differentiate by doing hgb electrophoresis; alpha = normal results; beta = slightly increased hemoglobin A2
75
Why do aortic valve defects cause issues with mucocutaneous bleeding?
They can cause an acquired loss of von willebrand factor due to shear stress on the multimers
76
Name two MPNs that are essentially diagnosed by excluding other MPNs
Essential Thrombocythemia and Primary Myelofibrosis (both can be JAK2V617F positive in 50%)
77
What are the criteria in which aplastic anemia is considered to be severe?
BM cellularity is less than 25% with 2 of: ANC \<500, platelet count \<20k, or absolute reticulocyte count \<20k
78
What mismatch repair (MMR) genes are often affected in Lynch Syndrome?
MLH1, MSH2, MSH6; there is a germline absence and then a somatic second hit occurs leading to the real problem
79
Brucellosis is acquired via \_\_\_\_\_\_\_\_\_\_\_. It often occurs as a systemic disease process; however, when occuring as localized disease it is called a \_\_\_\_\_\_\_\_\_
Unpasteurized milk; brucelloma--often as a granuloma in the liver (granulomatous hepatitis)
80
What is the initial diagnostic study for acute porphyria? What are some common sx?
A rapid urine screen for porphobilinoben; often severe abdominal pain, N/V, constipation and weird sensory abnormalities; red-tinged urine
81
What is the recommended therapy for recurrent Hodgkin Lymphoma especially with good response to salvage chemo?
Autologous SCT (i.e. recurrent then get something like DICE f/b BEAM followed by Auto-HSCT) (BCNU, Etoposide, Ara-C, Melphalan)
82
What is a typical cause of reactive monocytosis?
Autoimmune conditions; CMML can have a monocytosis and is an MDS/MPN overlap disorder with \>1000 monocytes or \>10% of the diff
83
What is the difference of a hemoglobin electrophoresis for alpha thalassemia minor and beta thal minor?
Alpha will have a normal hgb electrophoresis but beta thal will have elevated levels of hemoglobin A2; also, generally, hgb tends to be lower in beta thal minor than alpha minor
84
What therapy is often useful in lymphomas with bulky and/or painful LAD often denoted in the staging as "X" i.e. Stage IIBx?
Radiation therapy; loosely, bulky disease is defined as LAD \>10 cm but some disagree and have lower cutoffs; but ISRT is used
85
What did the TAPS study show for sickle cell patients prior to low to moderate risk surgery?
Transfuse to hemoglobin of 10 and showed a decrease in "clinically important complications"; keep in mind that going \>10 can increase viscosity and increase risk of thrombosis
86
MC cause of sore tongue, pancytopenia with macrocytosis and paresthesias?
B12 def can cause glossitis and present with pancytopenia and elevated LDH due to intramedullary hemolysis
87
What test is useful to identify iron deficiency anemia in a patient with concomittant inflammatory anemia?
Soluble transferrin receptor as it is elevated in iron deficiency but not inflammatory anemia
88
Patients with target cells (at least on boards) probably have some type of \_\_\_\_\_\_\_\_\_
Thalassemia either alpha or beta; if mild it is either alpha minor or beta minor and can only differentiate by doing hgb electrophoresis; alpha = normal results; beta = slightly increased hemoglobin A2
89
What test can diagnose most cases of vWD?
PFA-100 (platelet closure time, functional assay)
90
Patients on chronic immunosuppression are at increased risk of developing NHL, what are some viruses implicated?
Things like HTLV-1, EBV, HIV, and HBV + HCV can all be reasons for NHL in the setting of immunosuppression; same for PTLD
91
What should be done for patients with prostate CA who have elevated levels of PSA (\>0.2) after prostatectomy?
Should receive ADT as they have a high likelihood of harboring metastatic disease; prostate CA cells are androgen dependent but may become androgen-independent overtime
92
What is the most appropriate mgmt of patients with elevated ICP due to suspected primary CNS lymphoma (i.e. HIV pt with large periventricular mass)? Tx of PCNSL
High dose steroids first then brain biopsy (no use having tissue if youre dead!); 8-10 mg dex q6h and get Bx when stable; tx is HD MTX; of note, R-CHOP not great at getting past the BBB
93
Patients with CLL and severe and recurrent infxns can get IVIG to target an IgG level of \_\_\_\_\_\_\_\_\_; why do you need baseline Ig levels prior to tx?
\>500; need to rule out IgA def as it can cause anaphylaxis
94
A patient with normocytic anemia and a HR that is inappropriately low for the degree of anemia should prompt evaluation for \_\_\_\_
Hypothyroidism with TSH (didn?t know that it can cause normocytic anemia; also causes brady, would expect HR to be higher in anemic persons)
95
What is mixed (Type II) cryoglobulinemia without an identifiable secondary cause called?
Essential Mixed Cryoglobulinemia
96
What needs to be excluded to make a diagnosis of essential thrombocythemia?
Essentially need to exclude all of the other myeloproliferative neoplasms such as PV (rule out high hgb) and rule out CML i.e. BCR-ABL neg
97
What is the best test for pt with suspected B12 def with a low normal B12 level?
Methylmalonic Acid level (high in B12; note that homocysteine is high in both B12 and folate def); note that B12 \<200 very specific for def; \>300 unlikely but between 200-300 is where MMA useful
98
What is methemoglobinemia? Tx?
Hgb oxidized from the ferrous (2+) to ferric (3+); when all 4 hgb molecules are ferric then cannot bind O2; tx is with reducing agent IV methylene blue if high levels of methemoglobin are present (\>4g/dL) or if distributive shock (tissue hypoxia) is present
99
What is an anti-T cell antibody used for aplastic anemia?
Antithymocyte globulin (literally kills T cells); another lymphocyte ab is alemtuzumab but that kills T and B by targeting CD52
100
What is essentially the definition of platelet refractoriness? Who is at risk? What to do?
Assuming not ITP (bc that is different) it is an increase in platelets \<10k measured 10-60 min after after admin of plt on 2 occasions; multiparity and prior xfusion; HLA-matched platelet
101
What are 3 aspects of therapy for Chronic Granulomatous Disease?
Need to be on Bacterial PPx with Bactrim; Fungal ppx with Itraconazole (or posa/vori); and immunomodulatory therapy with IFN-Y
102
Which TKI is often preferred for Ph+ ALL?
Dasatinib, can even do monotherapy in older adults but generally HyperCVAD +/- R with Dasatinib (Sprycel)
103
True or false: early tx improves overall survival in follicular lymphoma? Translocation and pathophys?
No (Recall that ISRT may be used in early stage but generally just observe until sx or high FLIPI); recall that presence of centroblasts is the grade so grade IIIa is \>15/hpf with centrocytes present and \>15/hpf with solid sheets of centroblasts is IIIb and should be tx like DLBLC; t(14;18) translocation of BCL2 on 18 in front of enhancer elements of IgH on 14
104
What testing is indicated for patients who clinically appear to have a superficial thrombophlebitis?
US to evaluate for an actual DVT; sometimes need to monitor for progression as well with serial US
105
In the therapy of ALL who is considered AYA? Why does that matter?
Patients under 40 and that matters because can be treated with pegasparaginase containing regimens i.e. CALGB10403; adults get R-HyperCVAD if B cell w/ CD20; Hyper-CVAD if T cell (nelarabine if rel/ref), or HyperCVAD with or without R and Dasatinib if Ph +
106
What can be said about p16 immunohistochemistry in Head and Neck cancers?
Does not necessarily influence treatment at this time (2019) but does give information on prognosis as p16 positivity (surrogate for HPV infection) with locally advanced disease have significantly better prognosis
107
What is the classic timing of onset of drug-induced immune mediated thrombocytopenia?
Generally 5-10 days later; TMP-SMX is a known cause
108
The most likely diagnosis for a leukoerythroblastic blood smear in a pt with splenomegaly, anemia, and dacryocytes is \_\_\_\_\_\_\_
Primary Myelofibrosis (BM Bx is required) can have reticulin fibrosis, collagen fibrosis, or both; JAK2V617F identified in 50%; but remember, need to rule out other PMNs such as CML or burned out phase of something like PV
109
What is the treatment of aplastic anemia in older adults?
ATG and Cyclosporine (I think new data also has role for eltrombopag; but don?t forget that there is increased risk of marrow fibrosis with eltrombopag)
110
How is the treatment of acquired hemophilia different for patients with \<5 Bethesda Units of inhibitor vs. \>5?
If \<5 Bethesda units then can give Factor VIII concentrates to overwhelm the inhibitor by competitive inhibition; if \>5 then need a bypassing agent such as FEIBA or NovoSeven (aFVII)
111
What is the cause of anemia in pt with atypical lung infection with DAT+ and Anti-C3
Cold Agglutinin Disease especially if sensitive to cold weather
112
If a patient has a baseline prolonged PTT what is the most appropriate way to monitor unfractionated heparin?
Antifactor Xa levels; normally want 1.5 to 2.5x the control PTT but if baseline prolongation then target level of anti-Xa of 0.3-0.7
113
What are 3 general conceptual etiologies of hypofibrinogenemia?
Decreased production (i.e. ESLD), increased consumption (DIC), or issues with post-translational modification leading to absent or dysfunctional forms--afibrinogenemia or dysfibrinogenemia
114
Define limited and extensive stage SCLC
Limited stage involves only one hemithorax whereas extensive stage involves more than one hemithorax (radiation field)
115
The presence of red-brown macular skin lesions some of which darken and swell when rubbed in a pt with hx of flushing and anaphylaxis suggests what diagnosis?
Systemic Mastocytosis; rash is urticaria pigmentosa, sign is Darier sign
116
What are two drugs used to treat HIT and what cannot be used in renal failure?
Argatroban and bivalrudin but bivalrudin cannot be used in renal failure; fondaparinux also is one that may be used (remember to focus on platlet nadirs when evaluating thrombocytopenias)
117
The diagnosis suggested most strongly by microcytic anemia and normal iron stores with a normal RDW is \_\_\_\_\_\_\_
Thalassemia either alpha or beta; if mild it is either alpha minor or beta minor and can only differentiate by doing hgb electrophoresis; alpha = normal results; beta = slightly increased hemoglobin A2
118
What is the deal with parenteral anticoagulation with warfarin when bridging is done?
Need at least 5 days AND until the INR is greater than 2 for 24 hours (factor II and X levels take 5 days to decline sufficiently)--this is particularly important in HIT
119
What is haploinsufficiency?
When the haploid state of a gene is not sufficient to maintain the wild type phenotype; in other words, normally you may be heterozygous for something (loss of fxn of one allele) but still maintain normal; here loss of fxn of one allele still results in a disease state due to haploinsufficiency
120
Women with cancer of unknown primary site who present with abdominal carcinomatosis and ascites should be treated as if they have \_\_\_\_\_\_\_\_
Ovarian CA so Tx is with cytoreductive surgery followed by systemic chemo (i.e. Hyster + BSO, selective lymphadenectomy and appendectomy f/b platinum + taxane)
121
What was evaluated in the MAGIC trial?
The use of pre-op and post-op (neoadjuvant and adjuvant) chemotherapy + surgery vs. surgery alone for GE junction cancer w/ chemo being superior
122
How may a patient with macroangiopathic hemolytic anemia become iron deficient?
Patients with intravascular hemolysis will lose free iron in the urine but this does not happen with extravascular hemolysis
123
What is the treatment of high grade poorly differentiated neuroendocrine tumors of unknown primary site?
Basically, treat like SCLC w/ a platinum based chemo (i.e. cisplatin and etoposide)
124
What is the treatment of early stage head and neck cancer (stage I or II w/o LN involvment)?
Surgical resection vs. primary radiotherapy (would imagine if concommitant bad lungs would prefer RT d/t surgical risk)
125
What are the most appropriate first line treatments for symptomatic anemia in low risk MDS?
ESAs and transfusion support esp. if the EPO is \<500; HMAs are more for higher risk and elderly, if high risk and young can do Allo-SCT
126
Periorbital purpura worsened by valsalva maneuver is a relatively insensitive but fairly specific test for \_\_\_\_\_\_\_\_\_\_\_
AL amyloidosis; often has to do with adsorption of Factor X to the light chain; i.e. Raccoon Eyes
127
What is the preferred treatment for patients with acquired hemophilia with high levels inhibitor? What is the unit in which inhibitors are measured?
Activated Factor VIIa (Novoseven); Bethesda unit (one unit is enough to deplete one unit of factor by 50% after 2 hr incubation); so low inhibitor levels can be "overwhelmed" with regular factor rather than bypassing with FEIBA or NovoSeven
128
When would an allogeneic transplant be used for Hodgkin Lymphoma?
Not really used unless chemoinsensitive or relapsed after an auto SCT
129
What is the primary therapy of moderate to severe acute chest syndrome in sickle cell disease?
Exchange transfusion with a goal HbS \<30%; the exchange allows for getting rid of bad hgb while also not having issues with viscosity
130
What are the components of FOLFIRNOX? When is this used for pancreatic CA?
Folinic Acid, 5-FU, Irinotecan, and Oxaliplatin - used in metastatic and recurrent pancreatic CA with good performance status (i.e. s/p Whipple); gemcitabine alone ok for poor PS or in combo with nab-paclitaxel (Abraxane)
131
How long before standard and high risk surgeries should DOACs be stopped?
24-36 hours before standard risk; 2-4 days before high risk
132
What chronic transfusion dependent form of MDS often has a thrombocytosis? Tx?
5q minus syndrome; lenalidomide 10 mg po daily
133
What should be considered in a patient with CVID who develops night sweats and fevers?
NHL; patients with Common Variable Immunodeficiency like all immunodeficiencies have higher risk of NHL (often d/t viral stuff i.e. HTLV1, EBV, etc)
134
What are CD55 and CD59?
CD55 is Decay Accelerating Factor; CD59 is Membrane Inhibitor of Reactive Lysis; often with loss of PIGA gene
135
What is the CHINA mnemonic for hypereosinophilia?
Connective Tissue dz; Helminthic Infxn; Idiopathic (HES), Neoplasia, Allergies
136
What is the mineral deficiency that can manifest similarly to MDS or acute leukemia?
Copper; tx is with IV copper
137
The presence of vasculitis in the a patient with lymphoma and RF positivity with low C4 is consistent with this diagnosis \_\_\_\_\_\_\_\_\_\_
Cryoglobulinemic vasculitis--generally mixed type II where the antigen-ab complexes are polyclonal IgG and monoclonal IgM; MC assoc with HCV but also HBV, EBV, HIV and lymphoma
138
What other malignant disorder should be on the DDx aside from PV for an erythrocytosis?
Renal Cell Carcinoma due to ectopic EPO production; note the difference; EPO is low in PV but high in RCC; also look for back pain and hematuria
139
How would you differentiate Evans syndrome from TTP?
Evans syndrome will have the combination of ITP and AIHA so, like TTP will have anemia and thrombocytopenia; however, TTP is due to MAHA so will see schistocytes on smear rather than microspherocytes; also Evans is DAT positive whereas TTP is not
140
In an RCT known as ACTIVE W what was found with respect to aspirin and plavix vs. warfarin in pt with nonvalvular afib?
Warfarin was superior
141
What is the dosing for new DVT for rivaroxaban?
15 mg bid for two weeks then 20 mg daily (Recall that dabigatran and edoxaban require parenteral bridging)
142
What is the Mentzer index? In what situations is it useful?
MCV/Erythrocyte count; along with the RDW can help distinguish Fe def from thalassemia; normal RDW with Mentzer \<13 likely thalassemia but high RDW with Mentzer \>13 likely Fe def
143
What is suggested by a pt with normal platelet count and normal coagulation studies with personal and family history of mucosal bleeding?
Inherited platelet defect (as a general rule: mild plt defects can be tx with DDAVP and tranexamic acid or aminocaproic acid; if severe need plt transfusions prn)
144
What is standard surveillance for patients with colorectal cancer post operatively?
Follow up q3-6 months with CEA checks and CT C/A/P q year for 3-5 years; if a colonoscopy is done pre-op to look for synchronous primaries then can be done one year after then every 3
145
What are agnogenic myeloid metaplasia and myelofibrosis with myeloid metaplasia?
These are both synonymous with primary myelofibrosis; agnogenic means without known cause (idiopathic essentially); Metaplasia refers to the fact that the bone marrow is changing over to fibrotic tissue
146
What are the symptoms of hepatic sequestration?
A crisis situation in sickle cell disease whereby large numbers of RBC are trapped in the liver with RUQ pain, hepatomegaly, and severe anemia with a distributive shock; note splenic sequestration can occur in kids but rare in adults bc have infarcted the spleen
147
Which type of hemolysis is more likely to lead to iron deficiency: extravascular hemolysis or intravascular hemolysis and why
Intravascular because the free iron can be lost in the urine; in extravascular (i.e. spleen/liver) it is recycled via the RES
148
How does methylene blue work in methemoglobinemia?
Activates NADPH-dependent methemoglobin reductase system to rapidly reduce methemoglobin to hemoglobin
149
What are the 3 general scenarios by which a person can develop a hypereosinophilic syndrome? Usual Eosinophil cutoff?
Idiopathic HES; MPN HES with FIP1L1-PDGFRB or similar mutants; Lymphoproliferative HES due to production of IL-4 and IL-5 by the tumor; eosinophils \>1500
150
What is the primary therapy of a pt without hx of thrombosis who has PV? What if thrombosis?
Phlebotomy to decrease hct to 45% in men or 42% in women; can add hydroxyurea and aspirin as well as any necessary AC
151
What is the best mgmt for INR \>10 without bleeding in pt on warfarin? What does the evidence show?
Give 2.5-5 mg of vitamin K; it does NOT decrease bleeding rates but it does decrease the number of days to get to normal INR vs. holding warfarin only
152
What is the best test to quickly diagnose TTP?
A peripheral blood smear as it is a clinical diagnosis and you need the sx and thrombocytopenia and MAHA, an ADAMTS13 is NOT required for dx
153
Define MGUS? How often to monitor? How often to monitor if smoldering myeloma?
M-spike \<3 g/dL, \<10% plasma cells in BM, no CRAB; follow q6 months or so with labs; if smoldering myeloma then follow more like every 3 months; risk of progression to MM, Waldenstroms, or AL amyloidosis is 1% per year
154
What is the most likely etiology of thrombocytopenia in a patient who has cellulitis and has recently started vancomycin?
Drug induced (vancomycin) thrombocytopenia
155
What is the most appropriate therapy for reducing future strokes in sickle cell pts with hx of TIA or stroke?
Prophylactic RBC transfusion with exchange being more efficacious than simple xfusion though more invasive
156
An eosinophil count \>1500 with end organ involvement should prompt one to think of _________ as a dx
Hypereosinophilic syndrome
157
What is the standard treatment of DLBCL regardless of stage?
R-CHOP though with higher r-IPI scores (and double hit, BCL2 and MYC) may need Da-EPOCH-R or R-HyperCVAD etc
