Ocular granulocytic sarcoma (chloroma) with acute myelomonocytic leukemia in turkish children

Abstract

In a series of 166 leukemic children from Turkey, 56 had acute myelomonocytic leukemia (AMML). Seventeen boys and 3 girls presented with chloroma-like deposits (granulocytic or myeloid sarcomas) in the eye and orbit, all showing AMML on initial study of blood and marrow. The ocular lesions responded rapidly to antileukemic therapy. Laboratory studies of AMML cases revealed no cytogenetic or immune defects, and Epstein-Barr virus titers were normal. A group-specific (GS-3) antigen (type-C virus?) was identified in one patient by radioimmunoassay of orbital tumor extracts. It is not clear what factors contribute toward the myelomonocytic differentiation of leukemia and its localization in the eye and orbit, but spportunities for further study are enhanced by reports of a predisposition to ocular chloroma among leukemic children in Africa, Egypt, and Japan.

A prospective study of ocular manifestations in childhood acute leukaemia

“…This may appear before, after or concurrently with haematological manifestations of myeloid leukaemia. Biopsy may be necessary, especially when the underlying systemic disease presents first in the orbit and is otherwise unsuspected (Cavdar et al, 1978;Zimmerman & Font, 1975). The orbital mass of cells formed in myeloid leukaemia has been called granulocytic sarcoma.…”

“…This may occur as the terminal event of disease (Ridgway et al, 1976) and has been associated with a poor prognosis (Zimmerman & Font, 1975). The incidence of orbital granulocytic sarcoma has been reported to vary from 26.9% (Shome et al, 1992), 36% (Cavdar et al, 1978) to 46% (Davies & Owor, 1965).…”

“…The spectrum of ocular involvement in childhood leukaemia can be divided into three groups: (i) direct infiltration in orbit and ocular tissues (iris, choroid, retina, optic nerve) (ii) vascular abnormalities affecting the retina (intraretinal haemorrhages, white centered haemorrhages, cotton wool spots, macular haemorrhage, subhyaloid haemorrhage and vitreous haemorrhage) which reflect changes in haematological status and (iii) neuro-ophthalmic signs (papilloedema secondary to raised intracranial pressure and isolated cranial nerve palsies) of central nervous system disease (Rennie, 1992). The reports available on this subject in the literature (Ridgway et al, 1976;Cavdar et al, 1978;Robb et al, 1978;Novokovic et al, 1989;Stewart et al, 1989;Ohkoshi & Tsiaras, 1992;Humayun et al, 1992;Shome et al, 1992;Lo Curto et al, 1994;Mc Manaway & Neely, 1994) are either studies done at any time in the course of the disease or isolated reports or pathological studies. Ridgway et al (1976) reported 9% (59 of 657) of ocular abnormalities in children suffering from acute leukemia.…”

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.…”