Angiotropic large cell lymphoma with mononeuritis multiplex mimicking systemic vasculitis.

Abstract

Case report A 77 year old man was admitted for asthenia, weight loss, and fever (38-5°C), that had been present for two months before admission. Medical history included arterial hypertension and serous otitis media treated by myringotomy tube three years previously. One month before admission, skin lesions had been noticed on the left arm, forearm, and leg, then subsequently on the lower part of the abdomen and on the thighs. The lesions were successive and regressive within seven to 10 days. They appeared as inflammatory, infiltrated, and irregularly limited plaques a few cm in diameter. One of these was strongly suggestive of livedo reticularis. Initially, inflammatory arthralgias and myalgias were present. The patient then developed asymmetric sensorimotor neuropathy of the legs, involving initially the popliteal nerves, which progressively worsened. At the time of admission he also complained of bilateral testicular pain with local inflammation; this regressed within a few days.Routine haematological and immunological studies showed no abnormalities, except for lactate dehydrogenase, which was raised to 700 to 1000 IU/ml. Electromyography and nerve conduction studies of the legs showed an axonal sensorimotor neuropathy. The CSF was normal. Abdominothoracic CT and medullary MRI were normal. Histological examination of a skin biopsy showed a leucocytoclastic vasculitis; a muscle biopsy, a myelogram, and a bone marrow biopsy were normal.The neurological manifestations in the legs progressively worsened, and four months after the initial symptoms, neurological examination disclosed flaccid paraplegia, severe hypoaesthesia, perineal anaesthesia, urinary retention, and anal incontinence. His knee and ankle reflexes disappeared. He developed massive oedema with subsequent hypotension and profound hyponatraemia. Progressively, a central hypothyroidism occurred; triiodothyronine, thyroxine and TSH concentrations were very low, and FSH and LH concentra-

Skin manifestations of intravascular lymphoma mimic inflammatory diseases of the skin

“…Often the clinical findings are misinterpreted when the patient first consults a dermatologist. Common misdiagnoses are listed in Table 4 and include ‘thrombophlebitis’, ‘thrombophlebitis migrans’, ‘erythema nodosum’, ‘vasculitis’ and ‘livedo racemosa’ 51,56,61 . Associated findings such as fever, malaise, and elevation of lactate dehydrogenase and erythrocyte sedimentation rate may also mislead a dermatologist to suspect an inflammatory condition rather than a lymphoma.…”

Intravascular lymphomatosis presenting as an ascending cauda equina: conus medullaris syndrome: remission after biweekly CHOP therapy

“…As this form of lymphoma is potentially treatable and even curable when chemotherapy is initiated early,4 histopathological diagnosis is mandatory.…”

Intravascular lymphoma presenting as a longitudinally-extensive myelitis: Diagnostic challenges and etiologic clues

“…CNS manifestations include a subacute encephalopathy, dementia, cerebrovascular disease, seizures, cranial neuropathy (particularly cranial nerves VII and VIII), and myelopathy [5,6,[9][10][11]. PNS involvement is rare: a radiculopathy, polyradiculopathy, cauda equina syndrome, peripheral neuropathy, or myopathy have all been described [5,8,[12][13][14][15][16][17][18][19]. As in our case, a subacute onset progressive paraparesis due to spinal cord involvement may be the presenting manifestation of the disease [10,20,21].…”