You Make the Call
TRAINING and EDUCATION
Each month in “ You Make the Call ,” we ’ ll pick a challenging clinical question submitted through the Consult-a-Colleague program and post the expert ’ s response . But , what would YOU do ? We ’ ll also pose a submitted question and ask you to send your responses . See how your answer matches up to the experts in the next print issue .
This month , Martin S . Tallman , MD , weighs in on the treatment for a patient with myeloid sarcoma after induction .
Clinical Dilemma :
A 45-year-old woman presented with a painful 11 cm ovarian mass . It was surgically resected and found to be a myeloid sarcoma . Several surrounding lymph nodes were noted to contain similar findings . A bone marrow biopsy was negative for acute myeloid leukemia ( AML ). Cytogenetics and molecular markers were not obtained due to tissue fixation . The patient has completed chemotherapy with standard daunorubicin / cytarabine and has pancytopenia . A bone marrow biopsy was repeated and remains normal . How do I follow / assess this patient ’ s response ? What treatment should I give after induction ?
Experts Make the Call
Martin S . Tallman , MD Chief , Leukemia Service Memorial Sloan Kettering Cancer Center Professor of Medicine Weill Cornell Medical College New York , NY
In a large series from ECOG-ACRIN reporting , an analysis of more than 3,500 newly diagnosed patients with AML , the incidence of myeloid sarcoma ( MS ) was 23 percent and its presence was not found to be an independent prognostic variable . As was done in this case , whenever possible a biopsy of a soft tissue mass should be performed even when the marrow is involved . In the absence of a history of AML , the diagnosis can be difficult to establish . The t ( 8 ; 21 ) cytogenetic abnormality has been associated with MS as has inv ( 16 ), 11q23 , and t ( 8 ; 16 ) among others . The presence of isolated MS often heralds marrow involvement in approximately five to 12 months , so it has become routine to initiate systemic induction chemotherapy when the diagnosis is established . The optimal post remission therapy is not known . The cytogenetic or molecular genetic characteristics of this patient ’ s disease are not known , but would have been important for determination of risk and potentially for monitoring for minimal residual disease . If we consider that the patient has intermediate-risk disease , one could consider an allogeneic transplant if a suitable donor can be identified since it should provide the best anti-leukemia effect and her young age should minimize transplant-related mortality . However , its role in this setting is not established . The benefit of a cycle of consolidation before transplantation is not clear . However , depending on timing of transplant one might consider a single course of high-dose cytarabine , which should also penetrate sanctuary sites .
The role of radiation therapy is similarly not clear , but can be considered for residual bulky disease after consolidation chemotherapy depending on location . However , if there is no proof that such a combined strategy is better than chemotherapy alone . A screening lumbar puncture is reasonable as are periodic CT scans to follow the disease .
ADDITIONAL RESOURCE Bakst RL , Tallman MS , Douer D , Yahalom J . How I treat extramedullary acute myeloid leukemia . Blood . 2011 Oct 6 ; 118:3785-93 .
Consult a Colleague Through ASH
Consult a Colleague is a service for ASH members that helps facilitate the exchange of information between hematologists and their peers . ASH members can seek consultation on clinical cases from qualified experts in 11 categories :
• Anemias
• Hematopoietic cell transplantation
• Hemoglobinopathies
• Hemostasis / thrombosis
• Lymphomas
• Lymphoproliferative disorders
• Leukemias
• Multiple myeloma & Waldenström macroglobulinemia
• Myeloproliferative disorders
• Myelodysplastic syndromes
• Thrombocytopenias
Assigned volunteers (“ colleagues ”) will respond to inquiries within two business days ( either by email or phone ).
Have a puzzling clinical dilemma ? Submit a question , and read more about Consult-a-Colleague volunteers at hematology . org / Clinicians / Consult . aspx or scan the QR code .
* If you have a request related to a hematologic disorder not listed here , please email your recommendation to ashconsult @ hematology . org so it can be considered for addition in the future .
DISCLAIMER : ASH does not recommend or endorse any specific tests , physicians , products , procedures , or opinions , and disclaims any representation , warranty , or guaranty as to the same . Reliance on any information provided in this article is solely at your own risk .
Next Month ’ s Clinical Dilemma :
A 33-year-old female presents with sickle cell disease . Past hemoglobin electrophoresis shows double heterozygosity for Hb S and Hb C : HGB F 3.2 Hb S = 46.7 % Hb C = 50.1 %. Baseline hemoglobin is in the 10s . She has relatively mild disease clinically , with mild to moderate pain crises once every one to two years , but none in the last few years . The patient attributes this to having moved to Hawaii . ( She experienced more pain crises previously , when she lived at elevation .) Her most recent significant hospitalization was in 2009 when she had pneumonia , Hb of 5 g / dL with fevers , HAs , and required 4-5U PRBCs . She has been transfused ~ 10 times in her lifetime .
She is going to have bariatric surgery with a Roux en Y procedure . There are limited data published on how to manage these patients through such surgery , and we feel she should have exchange transfusion prior to surgery , though this is not available at our center or on the island of Hawaii . We do not have great experience with partial exchange . Please provide recommendations on how this patient should be managed .
How would you respond ? Email us at ashclinicalnews @ hematology . org .
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