Facial palsy as first presentation of acute myeloid leukemia

American Journal of Hematology 74:200–201 (2003) Facial Palsy as First Presentation of Acute Myeloid Leukemia Bhrigu Raj Sood,* Brij Sharma, Satish Kumar, Dalip Gupta, and Ashok Sharma Department of Medicine, Indira Gandhi Medical College, Shimla, H.P. India Granulocytic sarcoma or chloroma is a tumor composed of myeloblast or monoblast. These tumors may be found in any location of the body and occur in 5% of acute myeloid leukemia (AML) cases and are more common in pediatric patients. In this report we describe the case of a 27-year-old male who developed lower motor neuron seventh nerve palsy followed by swelling of the parotid gland. Fine-needle aspiration cytology (FNAC) of the soft tissue swelling revealed scattered blasts, and peripheral smear and bone marrow aspirate examination suggested AML. This is a rare presentation of a granulocytic sarcoma leading to a diagnosis of AML. Am. J. Hematol. 74:200–201, 2003. ª 2003 Wiley-Liss, Inc. Key words: acute myeloblastic leukemia; granulocytic sarcoma; chloroma; myeloblastoma; parotid; seventh cranial nerve palsy INTRODUCTION Granulocytic sarcoma is an uncommon manifestation of myelogenous leukemias in which focal masses of immature myeloid cells from the granulocytic lineage infiltrate bone and soft tissue. It is most common in the pediatric population and may present at any time in the course of the disease, either concurrently with the onset of leukemia or during a remission or relapse. Occasionally, this tumor may be the initial manifestation of AML and the appearance of the disease in the blood and marrow may follow weeks or even months later, presenting a diagnostic challenge. These tumors may be found in any location, especially skin, orbit, paranasal sinuses, bone, chest wall, breast, gastrointestinal, genitourinary, respiratory tracts, central nervous system, peripheral nerves, and lymph nodes [1]. CASE HISTORY A 27-year-old male presented to the oto-rhynology department with a 1-week history of a deviation of the angle of the mouth towards the left and inability to close the right eye. Clinical findings suggested lower motor neuron seventh cranial nerve palsy and he was treated along the lines of Bell’s palsy. A week later he developed swelling over the right side of the cheek and ª 2003 Wiley-Liss, Inc. pain below the right ear lobule and adjoining region and was examined in the medicine outpatient department. General physical examination was unremarkable except for the pallor. Local examination revealed a rounded, firm, discrete, tender swelling in the right parotid region measuring 5  5 cm. (Fig. 1). Neurological examination was normal except for the right infranuclear facial palsy (Fig. 1). Taste sensation on the anterior tongue was normal. The rest of the systemic examination was normal. Fine-needle aspiration was taken from the swelling that revealed infiltration by the monoblasts with thin, folded nuclei, fine chromatin, conspicuous nucleoli, and vacuolated cytoplasm, suggestive of monoblastic granulocytic sarcoma. A hemogram revealed anemia (Hb, 7 g%), platelet count of 80,000/mL, TLC of 18,000/mL, and differential count revealed 90% immature cells, most of which were blasts with lobulated nuclei, cytoplasmic vacuolation and lacy chromatin, suggestive of AML-M5. Bone marrow aspirate was also consistent with the diagnosis of AML-M5. *Correspondence to: Dr. Bhrigu Raj Sood, Kailash Bhawan, Near Children Park, Sanjauli, Shimla, H.P., India. 171006 E-mail: [email protected] Received for publication 16 June 2003; Accepted 16 June 2003 Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/ajh.10406 Brief Report: Facial Palsy and AML 201 mediastinum, prostate, bone, face, hard palate, oral cavity, genitor urinary tract including diffuse involvement of kidney or bladder, or other organs [3–9]. Such infiltration with leukemic blasts at diagnosis is characteristic of the monocytic subtypes (M4 and M5) [3]. These tumors may rarely precede evidence of systemic disease [2,3,6,7], but usually indicate widespread disease and require chemotherapy. When presenting at an extramedullary site, especially when preceding peripheral blood and bone marrow manifestations of leukemia, a misdiagnosis of histiocytic lymphoma may result. In questionable cases, other techniques including the naphthol-ASD-chloroacetate stain, touch imprints, immunoperoxidase stain for lysozyme, and electron microscopy should be utilized [6]. The role of immunohistochemical studies using antibodies against CD34 has been suggested for diagnosis of primary granulocytic sarcoma [10]. In these patients the presence in high concentrations of lysozyme, a marker for monocytic differentiation, may be etiologic in renal tubular dysfunction, which could worsen other renal problems that arise during the initial phases of therapy [3]. Fig. 1. Clinical photograph showing right seventh cranial nerve palsy and right parotid swelling (arrow), which turned out to be a monoblastoma and was the presenting feature of AML-M5 in this patient. With the final diagnosis of AML-M5 with granulocytic sarcoma of the right parotid gland with right seventh infranuclear facial palsy, the patient was started on induction chemotherapy with cytarabine and daunorubicin. Parotid swelling decreased in size and the patient was discharged. DISCUSSION Granulocytic sarcoma is an extramedullary tumor composed of myeloblasts and more differentiated cells, usually associated with acute myeloid leukemia. Chloroma, an old name for granulocytic sarcoma, is a clinical term. 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