Indian Pediatrics 2001; 38: 1199-1200  

Acute Myeloid Leukemia Presenting with Sudden Bilateral Proptosis as a Sole Manifestation

A 5-year-old male child presented with sudden (7 days) bilateral proptosis, which started with the protrusion of left eyeball followed by right eyeball on the very next day. This bilateral proptosis progressed rapidly to a grotesque form in the next 4-5 days. History of fever, vomiting, anorexia, weight loss or bleeding from any site was absent. Examination revealed, a conscious, markedly irritable child with normal vital parameters. Protrusion of both eyballs, more so of the left side and left sided facial palsy were the only other positive findings. Both eyes were almost totally blind. Other examination was normal.

Considering the sudden development of bilateral proptosis with left sided facial palsy in a previously healthy child, a cavernous sinus thrombosis was suspected and urgent CT scan cranium was done which was normal.

His Hb was 10.0 g/dl, TLC-30,000 cells/cu mm with P-60% and L-38% with normal BT and CT. Later peripheral smear examina-tion revealed acute myeloid leukemia (AML) of M4 type. Bone marrow examination confirmed the diagnosis.

Acute leukemias are the most common neoplasm in childhood accounting for 30% of all malignancies below 15 years of age. Usually, they present as nonspecific symp-toms due to derangement in hemopoietic system. Very rarely they present with extramedullary soft tissue mass due to tumor of leukemic cells known as ‘chloroma’ or ‘granulocytic sarcoma; as in our case(1). Almost any organ can be involved but eye is a common site. This type of presentation is most common with t(8:21) translocation and M4 and M5 type of AML(2).

Our case was unusual in the respect that sudden bilateral proptosis along with left sided facial palsy was the only presenting sign without any other evidence of acute leukemia, despite the involvement of hemopoietic system.

Ghanshyam Swami, Achla Arya, Balram Chowdhry,
Department of Pediatrics,
J.L.N. Medical College and Hospital,
Ajmer, Rajasthan, India
E-mail: [email protected]

References:

1. Shome DK, Gupta NK, Prajapati NC, Raju GM, Choudhury P, Dubey AP. Orbital granulocytic sarcomas (myeloid sarcomas) in acute nonlymphocytic leukemia. Cancer 1992; 70: 2298-2301.

2. Johansson B, Fioretos T, Kullendorff CM, Granulocytic sarcomas in body cavities in childhood acute myeloid leukemias with 11q23/MLL rearrangements. Genes Chromo-somes Cancer 2000; 27: 136-142.