High Risk Subgroup of Acute Myelomonocytic Leukemia (AMML) with Orbito-Ocular Granulocytic Sarcoma (OOGS) in Turkish Children

Abstract

Thirty-three patients presenting with orbito-ocular granulocytic sarcoma (OOGS) and acute myelomonocytic leukemia (AMML) were diagnosed in Turkish children from 1963 to 1983. OOGS, characterized by exophthalmos, chemosis and orbital masses, was observed in 33 (27.2%) of 121 AML patients compared with 41 children of AMML without ophthalmic tumors during the same period. Eye tumor and bone marrow aspirates were also studied under light and electron microscopies. The comparison of the hematological parameters did not indicate any statistical difference between the groups. Despite similar chemotherapy regimens administered to all patients, the mean survival time was 8.7 months in the OOGS group, which is significantly shorter compared to those without OOGS (28.6 months) (p < 0.01). These cases may be classified as a ‘high risk’ subgroup of childhood AMML.

Orbital granulocytic sarcoma and AML with poor prognosis in Turkish children

“…This localization of GS has generally been considered as an unusual or a rare finding in the Western world, in contrast to African and Asian countries. [2][3][4] Surprisingly, despite the presence of this important finding, no comment has been made on orbital localization, rather other EML involvements such as gingiva, CNS and skin, have been stressed. EML is a general term and the orbital form represents a subgroup, usually named either 'orbital chloroma' or OGS.…”

“…In our series, EML in the form of orbito-ocular or orbital granulocytic leukemia (OGS) was also more common in M4 subtype of AML. 2,3 OGS is a subgroup of granulocytic sarcoma (GS) that we have been interested in for many years. However, it seems to us that a very important finding, namely orbital localization of myeloblastoma or granulocytic sarcoma (GS) in 35 Dutch children is entirely overlooked.…”

“…EML is a general term and the orbital form represents a subgroup, usually named either 'orbital chloroma' or OGS. 2,3 Furthermore, there was no information about the race or ethnic origin and the socio-economic status (SES) of the patients, although ethnic diversity in The Netherlands is well known. Interestingly, in recent series of childhood GS reported from South Africa, GS occurred in 60% exclusively in black male children, mostly in orbital form, associated with t(8:21), indicating possible role of race and/or sex.…”

“…5 In our series, the OGS subgroup was from low SES within Turkey. 3,6 This was reflected by their T cell immunity (diminished delayed hypersensivity reaction and low T cell counts, etc) prior to their treatment. 6 The presence of OGS in 33 children associated with AML had unfavorably affected the prognosis in our series.…”

“…In fact, none survived in this group and the mean survival time of 8.7 months was significantly shorter (P Ͻ 0.01) compared with 28.6 months in the AML group without OGS, despite the fact that they had received similar chemotherapy regimens (Figure 1 dren should receive intensive chemotherapy, local radiotherapy to orbit and bone marrow transplantation (BMT), in order to have better survival. 3 Although intensive chemo-radiotherapy regimens have been used since 1990, and have improved the mean survival time in the OGS group of AML, overall prognosis has not changed significantly (unpublished data).…”