Benign Hematology
Questions discussed in this category
How would you approach anticoagulation in this situation? Does the presence of thrombocytopenia or hemorrhagic splenic infarcts change your manag...
For what duration and is there a preferred anticoagulation agent?
The patient takes frequent flights, with no prior history of VTE, and has already been taking measures for prevention including frequent ambulation, h...
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 ...
Would you anticoagulate for a fixed or indefinite duration? Would you recommend changing her contraception method?
Would you label this as intermediate or high risk and treat with prophylactic or full dose AC? What duration would you treat for?
Assume the patient has infrequent bleeding events every few years, with use of FIX replacement for acute bleeds only.
Presuming good medication adherence.
No prior hx of DVT/SVT. Negative LE doppler. Would you consider anticoagulating and at what dose, or favor close observation?
Do you consider starting Ursodiol? Do you perform routine abdominal ultrasound to monitor for cholelithiasis?
UpToDate recommends Hydroxychloroquine for all SLE patients, but neutropenia is sometimes ascribed to HCQ rather than the underlying disease. What lev...
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?
Caplacizumab is not FDA approved in pregnant patients, but has been used safely in isolated case reports.
When would you consider pomalidomide over bevacizumab?
Thrombophilia testing, including JAK2 is negative. When would you stop anticoagulation?
Would you consider testosterone therapy if he is otherwise asymptomatic?
Do the presence of gastric varices and use of anticoagulation change the way you think about using these agents?
If not, has your practice changed to use vWF replacement therapy more routinely for perioperative or acute bleeding management? Do you still perform D...
BMI>40
When do you stop luspatercept? How do you approach iron chelation therapy if the patient is currently on therapy?
Would you consider chronic RBC exchange versus HU?
Are there specific guidelines for managing this patient population?
Assume patient requires anticoagulation in the setting of acute thrombosis, with no need for IVC filter, but is approaching the end of her third trime...
What is your preferred anticoagulation in patients with BMI >50?
For B12 levels >2000-4000.
Hb at goal of 9-10
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...
Assume thrombolytics are not indicated. Do you favor early DOAC transition after 24-48 hours of heparin gtt or do you favor LMWH for 10-14 days follow...
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?
In light of ANNEXA-I RCT results: Connolly et al., PMID 38749032.
Are there specific mass features that would influence your treatment decision?
How often would you check labs during pregnancy or postpartum to monitor for worsening hemolysis?
Should adult hemoglobin targets be different than pediatrics?
Does this hold true when the patient has significant inflammation?
When would you phlebotomize patients with secondary hemochromatosis, such as due to NAFLD/cirrhosis?
HFE gene mutations in C282Y and H63D are negative.
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 ...
Aside from addressing the underlying case, is there a role for phlebotomy in secondary polycythemia such as in COPD or post-renal transplant erythrocy...
In the setting of requiring anticoagulation for atrial fibrillation and bioprosthetic valve, but also having severe vitamin K deficiency, would you st...
Is it different than early mobility that we always encourage?
Antifibrinolytic therapy alone +/- DDAVP?
E.g. young male, morbid obesity, with BMI >50
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?
Practice is variable in the community, with some hematologists frequently prescribing ESAs for severe anemia that is mostly inflammatory. Do the risks...
Anemia is secondary to menorrhagia. No gynecologic interventions were possible.
Level >60%. No family or surgical h/o of bleeding but heavy postpartum bleeding.
Hb <11, low iron saturation (<10%), high ferritin (>900), and low reticulocyte hemoglobin equivalent.
Is it only done when heparin is used in an emergency?
No transfusion dependence but has hemoglobin in the 8-9 g/dl range.
If so, for how long?
What is the work up and what is the duration of anticoagulation if used?
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.
Significant history of autoimmune diseases and DVT in family, recent PE/DVT for a month
Is patch or gel HRT with ASA prophylaxis a reasonable option after counseling? Or do you add a prophylactic DOAC?
Nonreliable INR, given hepatic coagulopathy
Is lifelong LMWH the anticoagulant of choice? Would DOACs be an option?
Although TIBC is negative acute phase reactant, would it be a better indicator of iron deficiency (in combination with ferritin)?
Also how would you manage this perioperatively?
Such as the case in which a patient is unresponsive to steroids, IVIG, TPO-agonist, rituximab, splenectomy, and even fostamitinib.
Often see bariatric surgery ordering these tests, is there any clinical relevance?
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.
The patient has no known history of autoimmune disease.
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...
Any particular labs or imaging indicated?
What can you recommend that might help keep them out of the hospital? Do you consider a program of exchange transfusions in this situation?
Would you bridge with enoxaparin 0.7 mg/kg/day in an ESKD patient, as described in a previous retrospective study (Pon et al., PMID: 24718051)?
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?
For example: In a patient presenting with left arm swelling and found to have a left cephalic vein occlusive thrombus on ultrasound
Should they be placed on routine EGD surveillance and if yes at what intervals?
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...
Does history of miscarriage affect your treatment decision?
Concordant low EPO level and bone marrow with megakaryocyte hyperplasia
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.
Do you use anticoagulation in cancer patients who develop an unprovoked superficial vein thrombosis?
If so, do you treat for a limited period of time or indefinitely?
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
Is SLE-related APLS managed differently in terms of anticoagulation?
MPN work-up negative
Specifically - patient who had mild aggregation defect with ADP which corrected in third trimester of pregnancy. Would it be appropriate or necessary ...
Although testing was not indicated, what do u do with these results?
Other hypercoagulability work up negative
E.g. low dose DOAC
Would the answer differ if the index event was arterial vs venous?
Previous provoking factors resolved (CVC, malignancy, etc)
Do you advise against combination OCPs?
If work-up is sent and the patient is found to have a persistently positive antiphospholipid antibody, particularly lupus anticoagulant, would you con...
What type, dosage, and duration of treatment do you use? How does your practice change when there is an inhibitor?
What is your preferred agent (DOAC, warfarin, enoxaparin), and is periprocedural bridging necessary?
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.
(Normal oxygenation and echo)
If so, is there one type of antibody that is more likely to cause this false positive test?
For example, if TSAT is less than 20% but ferritin is over 200.
For instance, evaluation by primary care and GI without other etiology for splenomegaly
Do you just use antibiotic prophylaxis if therapy is started prior to meningococcal vaccination?
Wu et al., PMID 34665865
Patient with high titer acquired FVIII inhibitor with no causative etiology. Bleeding is well-controlled on emicizumab, but inhibitor has failed...
i.e. similar to CKD anemia?
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
Is it time limited since it may have been triggered by the pregnancy or is it indefinite since it is APLS associated?
I.e., what constitutes well-controlled cancer, IBD, nephrotic syndrome, etc. What other diseases do you put in this category (obesity, autoimmune dise...
ALC<200
i.e. long car rides or plane rides
- Dialysis catheter used for hemodialysis- RIJ thrombus in dialysis catheter was incidentally found- Patient asymptomatic with no prior history of VTE...
CRP 39 mg/L (normal < 10) and normal ESR
APLA syndrome, obesity, non compliance ruled out and prefers another oral anticoagulation. Is higher dose of rivaroxaban or apixaban an option?
Bleeding disorder such as vWD
Presuming strong indication for ASA - eg history of NSTEMI
Should we stop checking factor VIII levels as part of thrombosis workup?
In the setting of no personal or family history and no other risk factors for thrombosis, is systemic anticoagulation warranted if local treatments (e...
How do you interpret elevations in antiphospholipid antibodies that are lower than Sapporo criteria?
(e.g. beta 2 glycoprotein IgM > 20 but <40)
e.g. K, Fya, s, etc
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
ET is JAK2+
If so, how long after diagnosis do you do so?
Recurrent thromboses despite therapeutic enoxaparin. Other hypercoagulable work-up negative.
In the absence of a VTE would you consider prophylaxis after a surgical procedure? Often non-hematologists order this testing but we are consulted for...
Would pegylated interferon be preferred?
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?
i.e. treatment-refractory PV, prior to progression to PMF or AML
Do you routinely check IgA anticardiolipin and beta-2 glycoprotein antibody IgA in your practice? And how would a positive result change your manageme...
e.g. DITP from eptifibatide after a cardiac intervention
How does cirrhosis and/or underlying thrombophilia affect your decision?
IVIG, TPO, or other agents?
Please comment on bridging.
Does VAF <1% make you want to do a BM biopsy for confirmation?
What should be done with a high level?
This is in the setting of a patient who is now on ruxolitinib with rising leukocytosis and thrombocytosis, but cannot be on aspirin due to recent blee...
For example, for outpatients or resource-limited settings with a moderate probability 4-T score (but low clinical suspicion), would you ever consider ...
e.g. in the setting of using prophylactic heparin in the past but now requiring therapeutic anticoagulation
Please also discuss the type and duration of anticoagulation.
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...
If so, what agent(s) do you prefer?
Hydroxyurea is demonstrated to reduce complications and improve long-term outcomes in severe genotypes HbSS and sickle beta0 thalassemia. What clinica...
What is your duration of anticoagulation?
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?
Is leukocytosis and thrombocytosis alone an indication for treatment?
Do you ever recheck JAK2/CALR/MPL/BCR-ABL? Would you recommend a bone marrow biopsy? Is cytoreductive therapy indicated?
Is there a role for aspirin or hydroxyurea? Do you perform phlebotomy, and if so, what goals?
What if the patient is no longer responding to steroids?
While low-dose aspirin for primary thrombosis prevention in aPL without APS is not typically recommended outside cardiovascular prevention guidelines ...
While building a trusting patient-physician relationship, what therapies could be discussed that may be aligned with naturopathic medicine? (i.e. L-gl...
Would appreciate expert opinion on when to reimage and when to restart anticoagulation depending on findings.
Is this a reason to start hydrea? Would you give oral iron after adequate control on hydrea?
Patient with ferritin level <1000 ng/mL and no evidence of end-organ damage
While there are many factors involving:- type (DVT vs PE, unprovoked vs provoked) and severity of venous thromboembolism (VTE) size- duration of antic...
If all work-up including peripheral flow, bone marrow biopsy, PET-CT returns negative, what additional management (if any) would be recommended?
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?
e.g. a genetic mutation picked up through a family member
Does transplant eligibility make a difference for management?
Do you recommend therapeutic phlebotomy to a certain Hct goal? Any strong evidence for thromboembolic risk related to erythrocytosis or if this is mit...
Is there any difference between anticoagulants in this clinical context (e.g. anti-Xa inhibitors vs warfarin vs LMWH)?
Is there a role for empiric antibiotics if there is history of opportunistic infection?
When do you consider initiating eculizumab?
What are your target goals during transfusion, either prophylactically or during acute complication? Does your management change between vaginal deliv...
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?
AstraZeneca may cause PF4 antibodies leading to vaccine-induced prothrombotic immune thrombocytopenia (VIPIT).
Assuming the patient is a candidate for all anticoagulation options (no mechanical valve, antiphospholipid syndrome, patient-specific contraindication...
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...
While the CKD population is at high thrombotic and bleeding risk, would you consider anticoagulating a patient prophylactically if they had a history ...
The patient who has not responded to steroids, rituximab/bendamustine, and splenectomy?
In a patient with severe factor XII deficiency with a baseline prolonged PTT, what is the best method to monitor their ACT or heparin levels when on c...
Would you consider high-dose dexamethasone (deliberating adverse effects of antenatal steroids) or move to next-line therapies?
Has your medical practice taken any steps either in community outreach or within the clinic to show support for this medically vulnerable population, ...
For a patient with low ferritin, but high TSAT?
Ref: EINSTEIN-CHOICE and AMPLIFY-EXT
Would you approach this differently in patients with inherited thrombophilias?
For example, in cold agglutinin disease or AIHA, antibodies can be detected via DAT, but are often not observed on assays for monoclonal gammopathies.
During the Stimate recall, how are we performing DDAVP challenges for newly diagnosed hemophilia A or von Willebrand disease?
In low-risk patients (age < 40) or patients with very obvious causes of blood loss or iron deficiency (menorrhagia, pregnancy), do you routinely pe...
Are there effective therapy options that do not include steroids?
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?
Such as a patient who cannot tolerate oral iron and has had a anaphylaxis to intravenous iron
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
For example, a patient on cytoreductive therapy and aspirin BID, but suffers an arterial event, or a patient who is already on therapeutic anticoagula...
In a patient with a medical or personal indication to induce oligomenorrhea/amenorrhea, how would you manage OCP therapy if a patient develops a VTE d...
If autoimmune neutropenia already suspected, is this test informative or unnecessary?
What is the lowest level you have seen with uncomplicated or complicated crises?
Do you go straight to TMPRSS2 genetic testing or what other lab testing (e.g. hepcidin) could be helpful?
If there are a low-risk patients who can be monitored, how would you do so?
If unprovoked, would you consider stopping anticoagulation?
Should we use a different dose in critically ill patients in the ICU? When should we consider intermediate-dose anticoagulation?
Is there a preferred strategy of transfusional support versus reduced-dose anticoagulation during the duration of thrombocytopenia?
Rituximab has entered the treatment algorithm in the first-line setting for the initial treatment of TTP. However, in remission, the role of adjuvant ...
What if this was "triple-negative" antiphospholipid syndrome?
Does recent iron intake affect the iron panel, and if so, do you often order fasting labs in practice?
Does your opinion change based on the specific underlying inherited thrombophilia? The original case was a central retinal artery occlusion in a patie...
Especially in a patient without hemophilia or thrombosis history?
Would you offer this routinely or only if specific complications arise, such as distal emboli?
In patients who are intolerant to hydroxyurea, anagrelide and interferon
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