A 21-year-old male with no significant medical history presented with complaints of diplopia of three-week duration which resolved after treatment with steroids. After the initial encounter, patient presented with one week history of left sided facial droop indicative of facial nerve palsy. Extensive neurological work-up including lumbar puncture and imaging were unrevealing, which led to a diagnosis of isolated sixth and seventh cranial nerve palsies. During the same period, progressively worsening right upper quadrant abdominal pain and jaundice prompted him to come to the emergency department for further evaluation. Pertinent laboratory data included AST 67 (15-37 U/L), ALT 102 (U/L), alkaline phosphatase of 973 (45-117 U/L), total bilirubin of 6.8 (0.2-1.0 mg/dL) and direct bilirubin of 5.4 (0.0-0.2 mg/dL). He was also found to have leukocytosis of 16000 and platelets of 37000. Peripheral smear showed myeloblasts. Initial evaluation for obstructive jaundice included an abdominal ultrasound, which showed a mass-like structure in the gallbladder, dilated common bile duct (CBD) and intrahepatic biliary dilatation. A computerized tomography of the abdomen did not add further diagnostic information. This led to an endoscopic retrograde cholangiopancreatography being performed with placement of a CBD stent and eventual cholecystectomy. Pathology from the gall bladder revealed granulocytic sarcoma. Simultaneous work-up of hematological abnormalities, including a bone marrow biopsy, revealed 60% myeloblasts consistent with acute myeloid leukemia (AML) with t(8;21) translocation. He was started on induction chemotherapy.
Discussion:
Chloroma or granulocytic/myeloid sarcoma is a rare extra myeloid manifestation of AML. It presents as a mass lesion outside the bone marrow and represents a tumor composed of leukemic cells. AML is known to precede, accompany or develop after the occurrence of chloroma. It is more common in patients with t(8;21) translocation. When associated with AML, the diagnosis is usually straight forward, however, in isolated chloromas it is frequently misdiagnosed as a lymphoproliferative disorder. Central Nervous System (CNS) involvement in AML can be due to meningeal infiltration, epidural or parenchymal myeloblastomas (chloromas), or leukostasis. Occasionally patients can have CNS involvement with negative cerebrospinal fluid cytology.
Conclusions:
Chloroma is a rare occurrence but may be the initial manifestation of AML. A high degree of suspicion is required for diagnosis and recognition is critical to institution of appropriate therapy and prevention of complications. Isolated chloromas also deserve initiation of treatment as the incidence of AML in these patients is very high. It is important to be aware of the possibility of a systemic disease and have a broad differential diagnosis when patients present with multi-organ involvement.