Granulocytic Sarcoma (GS) as First Manifestation of AML.

Granulocytic sarcoma (GS), or chloroma is an extramedullary tumor of immature myeloid cells which occurs throughout the body, often associated with acute myeloid leukemia (AML) or in the accelerated phase of chronic myelogenous leukemia, but rarely as the first manifestation of AML, preceding the onset in marrow and blood by months or years[1, 2] Herein, we report GS of lymph nodes presenting as first manifestation of AML

Case Report

A 53 years old male presented with recurrent episodes of epigastric abdominal pain without history of trauma or infections, Physical examination was normal. Laboratory examination showed WBC 5.2, ANC 4.4, Hemoglobin 13.6 gm/dl, PLT.306, 000 and Retic 1.8. Computerized Tomography (CT) of abdomen and pelvis showed extensive abdominal and pelvic lymphadenopathy, gastric wall thickening and prominence of the pancreatic head versus peripancreatic lymphadenopathy. Subsequently Esophagogastroduodenscopy and colonoscopy were both negative for malignancy. Peripheral smear showed normal RBC, and no myeloblasts. Laparoscopic retroperitoneal lymph node biopsy revealed granulocytic sarcoma, with immunohistochemistry positive for CD43, CD117, CD68 and MPO

Bone marrow biopsy and aspirate were performed, showing increased M: E ratio, increased blasts (29%), with flow cytometry reporting positivity for CD13, CD33, CD34, CD117, and HLA-DR supporting the diagnosis of AML. Chromosomal analysis including Fluorescence in situ hybridization studies (FISH) showed no evidence of chromosomal abnormalities. Patient continued to have epigastric pain radiating to the back without fever, night sweats or weight loss.

He was treated with standard induction chemotherapy with Ara-c (7 days) and Idarubicin (3days), resulting in complete remission with resolution of the abdominal and pelvic lymphadenopathy.

Patient is currently receiving consolidation therapy with high dose Ara-c

Conclusion

Granulocytic sarcoma is a rare disease. The overall prognosis is poor. However, AML-type chemotherapy appears to improve survival.