This month, Martin S. Tallman, MD, weighs in on the treatment for a patient with myeloid sarcoma after induction.
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.
Bakst RL, Tallman MS, Douer D, Yahalom J. How I treat extramedullary acute myeloid leukemia. Blood. 2011 Oct 6;118:3785-93.
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I would recommend allogeneic stem cell transplantation if she has a match.
Bruce Raphael, MD
NYU Langone Medical Center
New York, NY
I would follow her periodically by checking SPEP, serum free light chain, CBC, and CMP. I would maybe ultrasound the pelvis periodically, then just watch and follow.
Manal Robin-Hanna, MD
Cancer Centers of South West Oklahoma
I would proceed with a bone marrow transplant after assuring remission with CT or PET/CT. If no matched siblings, I would consider an autologous bone marrow transplantation.
Yusra Al Awami, MD
King Faisal University
I would give induction, followed by consolidation (AML protocol). A CT scan could be done to assess the response.
Abdul Hameed, MBBS, MD
Shaukat Khanum Memorial Cancer Hospital & Research Centre
I would recommend salvage therapy followed by BMT.
Kulumani Sivarajan, MD
Joliet Oncology-Hematology Associates
Perform PET-CT scan and type siblings for possible match. I would then do three cycles of HiDAC.
Thomas Hyde, MD
Kaiser Permanente Rock Creek Medical Oncologists
Bone marrow transplant ASAP.
Anastasia Skandali, MD
To observe and consider bone marrow transplant.
I would give four cycles of HiDAC consolidation.
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.
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