Labs with normal PT, but prolonged PTT (47 sec, ULN 40 sec) that does not correct on immediate mix. Lupus anticoagulant negative (DRVVT and hexagonal ...
Assume the patient has infrequent bleeding events every few years, with use of FIX replacement for acute bleeds only.
Presuming good medication adherence.
Do you consider starting Ursodiol? Do you perform routine abdominal ultrasound to monitor for cholelithiasis?
After what time period would you consider adding a second iron chelator?
Do you discuss the risk of sickle cell crises with G-CSF? What about for patients with sickle cell trait?
Thrombophilia testing, including JAK2 is negative. When would you stop anticoagulation?
If not, has your practice changed to use vWF replacement therapy more routinely for perioperative or acute bleeding management? Do you still perform D...
Would you consider chronic RBC exchange versus HU?
Are there specific guidelines for managing this patient population?
No clear inciting etiology found.
Would you consider dexamethasone +/- cytokine blockade (IL-1, IL-6, or IFN-g)? What do you think about the data for...
Are the INRs reliable? In what scenarios would you not recommend POC INR use for warfarin monitoring?
Are these typically covered by insurance? Are t...
Do social or economic factors (i.e., relative cost of acquiring LMWH, the patient being injection averse) affect your decision to use DOACs?
Do you s...
Is there a factor XI goal that you target? Would you consider FVIIa products instead?
Should adult hemoglobin targets be different than pediatrics?
Does this hold true when the patient has significant inflammation?
Patients sometimes ask for annual urinalysis and ultrasound to monitor, but it is unclear if this is indicated.
Is a BM biopsy a must when there is skin involvement?
If tryptase level is mildly elevated but less than 20 would you recommend a BM biopsy?
The ASH 2020 guidelines have "recommended that adults with HbSS or HbSβ0 thalassemia be screened at least once for silent cerebral infarcts even ...
Antifibrinolytic therapy alone +/- DDAVP?
Ferritin >2200, TSAT >80%
There are various formulations of intravenous iron; each with varying costs, test dose requirement, elemental dose, and number or time of infusions ne...
While follow up ultrasound is not usually recommended in provoked DVT, it often is done either for other reasons or by other physicians. Would this in...
How would you counsel the patient if he/she wants to continue on steroids?
Common thought is that FVIII may be used for differentiating coagulopathy in liver disease (normal to increased, from reduced clearance of VWF/FVIII) ...
I.e. platelet count <30. Would your management change if HIT were only suspected rather than confirmed?
Anemia is secondary to menorrhagia. No gynecologic interventions were possible.
E.g. Normal F8, VWF activity ~50%, VWF antigen ~100%, ratio 0.5 sent in a patient with positive bleeding history
Is there any clinical significance t...
Specifically, asymptomatic subsegmental PE diagnosed within a month from planned bilateral mastectomy for breast cancer.
Although TIBC is negative acute phase reactant, would it be a better indicator of iron deficiency (in combination with ferritin)?
Such as the case in which a patient is unresponsive to steroids, IVIG, TPO-agonist, rituximab, splenectomy, and even fostamitinib.
Would you consider low dose indefinite anticoagulation in any scenario? Any difference in approach between hematological malignancy and solid tum...
No prior thrombosis; no family history of thrombosis. As per endocrine, the only useful therapy for the osteoporosis is estrogen.
Would it change your management if the thrombus was symptomatic? Or if larger >3 cm?
For example, a patient with a bone marrow biopsy that shows normocellular bone marrow. Prior management with leflunomide and HCQ with neutropenia attr...
If hydroxyurea modifies the course of sickle cell disease by increasing fetal hemoglobin, is there any benefit to using it in patients with fetal hemo...
Would you give a trial of IST first or immediately refer for SCT if the patient has matched siblings?
ADAMTS13 level <5%
Inhibitor level <1
E.g. pulmonary embolism, portal vein thrombosis, cerebral venous sinus thrombosis
If so, what platelet count threshold would you use? Would age impact your decision? Would you do a bone marrow to rule out primary MPD in adults if th...
That is, extra copy of one of the alpha genes resulting in an atypical alpha thalassemia for the patient, and beta thalassemia heterozygous carrier fo...
Please comment on temperature recommendations and role of exchange transfusion.
While L-glutamine has minimal side effects and would likely be added after hydrea, how do you decide between the use of voxelotor and crizanlizumab?
In the absence of concerning bleeding or thrombosis, family history of coagulopathy, current anticoagulant use, or malnutrition
Although testing was not indicated, what do u do with these results?
Other hypercoagulability work up negative
Would the answer differ if the index event was arterial vs venous?
Do you advise against combination OCPs?
What type, dosage, and duration of treatment do you use? How does your practice change when there is an inhibitor?
What would you recommend within the first year after ATG and thereafter if still maintained on cyclosporine?
Ferritin uptrending >1000, no additional lab abnormalities. HFE wildtype. Too young for age-appropriate cancer screening. Asymptomatic except fatig...
Is thrombotic risk too significant? Patient failed OCPs.
For example, if TSAT is less than 20% but ferritin is over 200.
Do you just use antibiotic prophylaxis if therapy is started prior to meningococcal vaccination?
Patient with high titer acquired FVIII inhibitor with no causative etiology. Bleeding is well-controlled on emicizumab, but inhibitor has failed...
The patient has no personal history of VTE, but has positive family history of VTE.
Any prophylactic anticoagulation options?
How would manage...
For instance, if clonal hematopoiesis of indeterminate potential (CHIP) suspected
I.e., what constitutes well-controlled cancer, IBD, nephrotic syndrome, etc. What other diseases do you put in this category (obesity, autoimmune dise...
i.e. long car rides or plane rides
CRP 39 mg/L (normal < 10) and normal ESR
Presuming strong indication for ASA - eg history of NSTEMI
Should we stop checking factor VIII levels as part of thrombosis workup?
(e.g. beta 2 glycoprotein IgM > 20 but <40)
How would the approach differ if the patient had a significant bleeding phenotype vs only minor bruising and mucosal bleeding?
Last rituximab 6 months ago
If so, how long after diagnosis do you do so?
FVL heterozygotes are often treated similarly to the general population. Aside from avoiding other VTE risk factors, are there situations where prophy...
Are there particular clinical scenarios that would affect your decision?
e.g. DITP from eptifibatide after a cardiac intervention
IVIG, TPO, or other agents?
Also is there value in using voxelotor for the purpose of reducing hemolysis, and if so what parameters do you use to determine when to initiate voxel...
Is there a particular sequence you would adjust contributing antirejection or antimicrobial medications? Is the use of G-CSF appropriate and at what c...
Hydroxyurea is demonstrated to reduce complications and improve long-term outcomes in severe genotypes HbSS and sickle beta0 thalassemia. What clinica...
What if the patient is triple-positive or has continued seropositivity on repeat lab testing? What is the appropriate interval of monitoring and does ...
Provoked or unprovoked VTE: Do you use D-Dimer (or even repeat imaging to reassess residual clot) in any capacity to guide anticoagulation duration? E...
Have you used anticoagulants other than coumadin? Or is that the only appropriate agent given monitoring is based on PT/INR?
While building a trusting patient-physician relationship, what therapies could be discussed that may be aligned with naturopathic medicine? (i.e. L-gl...
Does manipulation of blood products (leukoreduction, irradiated) affect your approach? There is growing evidence that routine premedication (at least ...
While this is a known risk factor for venous [Meijers et al NEJM 2000] and potentially arterial [Yang et al, Am J Clin Pathol 2006] thrombosis, it is ...
Are there major differences in standard conditioning and/or GVHD prophylaxis regimens utilized?
Does the non-relapse mortality different significantl...
How often do you monitor ADAMTS-13 levels off therapy?
For example, do we prefer one regimen over the other in patients with a bleeding history or who have relapsed after a lengthy remission?
Is there a role for empiric antibiotics if there is history of opportunistic infection?
While benefits outweigh known harms and limited data, do you worry about vaccination in patients with a history of or active autoimmune cytopenias (e....
How long after eculizumab infusion would you wait before giving blood transfusions?
While thrombophilia testing is not routinely recommended prior to starting OCPs, how about after the development of a VTE?
Especially in a triple-positive patient with an acute ischemic stroke who may have urgency for anticoagulation with high bleeding risk and severe thro...
The patient who has not responded to steroids, rituximab/bendamustine, and splenectomy?
Has your medical practice taken any steps either in community outreach or within the clinic to show support for this medically vulnerable population, ...
During the Stimate recall, how are we performing DDAVP challenges for newly diagnosed hemophilia A or von Willebrand disease?
Given the high cost of eculizumab, are there patient characteristics that inform which patients, if any, should be on indefinite therapy versus a time...
How do you manage an uncomplicated DHTFR versus a hyperhemolysis?
For example, a patient with hypogonadism unless it matters which organ is involved. Are other markers of iron storage useful in guiding therapy?
Presented at ASH: FLIGHT trial
If autoimmune neutropenia already suspected, is this test informative or unnecessary?
What is the lowest level you have seen with uncomplicated or complicated crises?
Is there a preferred strategy of transfusional support versus reduced-dose anticoagulation during the duration of thrombocytopenia?
What if this was "triple-negative" antiphospholipid syndrome?