Infra-Diaphragmatic Gray Zone Lymphoma Presenting with Paraneoplastic Neurological Syndrome

Case Report

Ann Hematol Oncol. 2017; 4(6): 1157.

Infra-Diaphragmatic Gray Zone Lymphoma Presenting with Paraneoplastic Neurological Syndrome

Torres J1,4, Hawkes EA1,2,3*, Hosking P1 and Bladin C1,2

¹Department of Oncology, Eastern Health, Australia

²Monash University, Australia

³Olivia Newton John Cancer Centre, Austin Hospital, Australia

4Goulburn Valley Health Australia

*Corresponding author: Hawkes EA, Department of Oncology, Eastern Health, 8 Arnold St, Box Hill, Victoria, Australia

Received: May 02, 2017; Accepted: May 31, 2017; Published: June 22, 2017


Paraneoplastic neurological syndromes (PNS) are well-documented phenomena in classical Hodgkin lymphoma (cHL). Cerebellar degeneration syndrome, motor neuron disease, myasthenia gravis, stiff person syndrome, chorea and ataxia have all been described. They are however, to our knowledge, unreported in gray-zone lymphoma (GZL). GZL is defined as unclassifiable B-cell neoplasm with features intermediate between diffuse large B-cell lymphoma (DLBCL) and cHL according to the 2008 World Health Organization. The majority of PNS fail to improve with treatment of the underlying malignancy.

Keywords: Gray zone lymphoma; Paraneoplastic syndrome; ABVD chemotherapy; Non-Hodgkin lymphoma

Case Presentation

A previously healthy 57-year-old man presented to the emergency department with three weeks of progressive urinary retention and rapidly progressive bilateral lower limb weakness causing decreased mobility, on a background of 15 kg’ weight loss over two months. He denied any infective symptoms, headache or neck stiffness.

The patient was a current smoker and consumed excess alcohol but had no significant medical conditions and no regular medications. Family history included colorectal cancer, in his father, aged 70 [1-3].

Neurological examination revealed bilateral lower limb weakness in an upper motor neuron muscle distribution, hypertonia, hyperreflexia, bilateral spontaneous clonus, and up-going plantar responses consistent with spastic paraparesis. Sensation remained intact.

Initial investigations revealed normal full blood examination, biochemistry, lactate dehydrogenase, C-reactive protein, Erythrocyte Sedimentation Rate, vitamin B12 and folate. The gammaglutamyltransferase and alanine aminotransferase were elevated, (230 IU/L [ULN: 71] and ALT 118 IU/L [ULN: 40] respectively). HIV and syphilis serology were negative. Magnetic resonance imaging (MRI) of brain and whole spine excluded a space-occupying lesion at any level, intracranial demyelization, spine infiltration and myelitis.

Cerebrospinal fluid (CSF) analysis revealed elevated lymphocytes (15×106/L) but normal polymorph counts and biochemistry, negative culture and cytology. Flow-cytometry failed to reveal abnormal clonal population. Nerve conduction studies were also unremarkable. Paraneoplastic-associated antibodies including anti-Yo, anti-CV2, anti-Glutamic Acid Decarboxylase, anti-Hu and anti-Ri were all negative. Computer Tomography (CT) of neck, chest, abdomen, pelvis demonstrated only prominent left iliac lymph nodes (<10 mm) but no other abnormalities (Figure 1B).