CASES OF EXTRAMEDULLARY MYELOID LEUKEMIA AT THE 1ST DEPARTMENT OF INTERNAL
POSTER 43
MEDICINE, SEMMELWEIS UNIVERSITY IN HUNGARY
Ilona Tarkanyi1, Alexandra Raska2, Julia Gaál-Weisinger1, Anna Rakonczai1, Zsolt Nagy1 and Judit
Demeter1
(1)1st Department of Internal Medicine, Semmelweis University, Budapest, Hungary
(2)3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary
Introduction:
Extramedullary leukemia is a rare manifestation of acute myeloid leukemia that can either be
isolated or can present with prior or concomitant leukemic bone marrow infiltration. Extramedullary
manifestation as the primary disease site is often a challenge, and can lead to delay in diagnostics
and treatment.
Aim:
We evaluated patients treated between 2005 and 2019 with extramedullary (EM) leukemia at the
1st Department of Internal Medicine, Semmelweis University in Hungary in order to review treatment
and disease outcomes as well as problems experienced during diagnostic workup.
Results:
In the previous 15 years only 4 cases occured, where bone marrow biopsy revealed isolated EM
leukemia; localizations were skin, central nervous system, and ovary. 7 cases presented with
extramedullary leukemia (skin, lymph node, soft tissue, breast, peridural and orbital) but
concomitant bone marrow infiltration was present. Further 4 patients with antecedent
myelodysplastic or Philadelphia-negative myeloproliferative disease showed first sign of blastic
transformation in form of skin, soft tissue or tonsillar myeloid sarcoma. Age of the patients at
diagnosis was 22 to 81 years.
10 patients were treated with high-dose induction chemotherapy, and 5 received palliative
chemotherapy based on their age and concomitant diseases. 6 patients treated with curative
intention achieved remission, 4 died during induction therapy. 3 patients in remission died during
early relapse, 1 deceased due to complications of allogeneic stem cell transplantation. 2 young
females- 23 and 44 years at presentation- are still in remission 15 and 9 years after high-dose
therapy- neither of them required stem cell transplant. As a consequence, fertility of the then 23-
year-old woman was preserved and she gave birth at the age of 36.
Conclusion:
Although the low number of cases does not allow for statistical evaluation, few conclusions can be
drawn from our observations. Diagnosis with extramedullary disease presenting other than
cutaneous leukemia had an acceptable diagnostic workup, whereas patients with mainly skin
presentation had a 3-month time delay on average. The latter was often due to histological
misdiagnosis- initially reported a cutaneous lymphoma- and needed hematopathological revision.
Risk assessment of EM leukemia and decision to proceed to allogeneic stem cell transplantation
requires clinical, cytogenetic, and molecular features to guide the decision. As seen in our surviving
patients, cases with favorable genetic features can achieve long term survival with chemotherapy
alone, without long term consequences of a stem cell transplant.