Monoclonal gammopathy (IgA: lambda) after autologous T-cell-depleted bone marrow transplantation in a patient with non-Hodgkin''s lymphoma

We present an unusual case of sphenoid sinus mucocele with severe intracranial extension, which was diagnosed and treated at the AHEPA General Hospital, University of Thessaloniki, Greece. The patient was a 54-year-old female who presented with endocrine, nasal and ophthalmological symptoms, and headache. She was successfully treated by surgery using an intranasal approach. While presenting this case, we briefly review the literature on the subject of sphenoid sinus mucoceles.     

Confocal microscopic localization of anti-myelin-associated glycoprotein autoantibodies in a patient with peripheral neuropathy initially lacking a detectable IgM gammopathy

AIMS AND BACKGROUND: The neutropenia induced by six courses of an intensified FEC regimen is expected to be checked by early primary administration of G-CSF which is stopped eight days before the next chemotherapy course. Less information is available about megakaryocytic and erythroid toxicity over six courses. METHODS AND STUDY DESIGN: Sixty-six consecutive patients with metastatic breast cancer completed six courses of a randomized treatment with two FEC regimens administered every 21 days, in which 600 mg/m2 of cyclophosphamide and 5-FUwas associated with 60 or 120 mg/m2 of epirubicin (60FEC, 35 patients, vs 120FEC, 31 patients). 120FEC was supported by early primary G-CSF (days 4 to 13). Blood counts were obtained seven times during each course. RESULTS: The non-hematologic toxicity over 364 courses was similar in 60FEC and 120FEC. No cumulative hematologic toxicity was observed for white blood cells (WBC) and platelets (PLT), while for hemoglobin (Hb) a somewhat higher cumulative toxicity was observed with 120FEC than with 60FEC. WBC, PLT and Hb grade III-IV toxicity occurred in 40.1% and 45.6% (P=ns), in 23.1% and 0.8% (P <.0001) and in 15.6% and 3.0% (P <.005) of the two regimens, respectively. There were no febrile or hemorrhagic episodes. The epirubicin relative dose intensity delivered was 1.95 in 120FEC with respect to 60FEC. CONCLUSIONS: Our G-CSF schedule permitted to deliver six courses of 120FEC without any clinically relevant side effects. Grade III-IV leukopenia was similar with 120FEC and 60FEC, while grade III-IV thrombocytopenia and anemia occurred more often with 120FEC than with 60FEC.     

Proliferative glomerulonephritis in monoclonal gammopathy

A 62-year-old white male suffering from plasmacytosis with monoclonal gammopathy developed gastrointestinal bleeding, microscopic hematuria, proteinuria and progressive azotemia. A renal biopsy demonstrated capillary wall thickening, mesangial hyperplasia, crescent formation and subepithelial humps. Serum immunoglobulin analysis showed IgG of lambda type. Serum complement, ANA, ASO titer and cryoglobulin were normal. It is suggested that proliferative glomerulonephritis with subepithelial humps should be added to forms of renal pathology that can be seen in plasma cell dyscrasias.     

Two patients with idiopathic pulmonary fibrosis and monoclonal gammopathy

A 67-year-old man and a 70-year-old man were admitted to our hospital because of dyspnea and dry coughing. Chest X-ray films showed bilateral reticulonodular shadows in the middle and lower lung fields. Specimens were obtained by open lung biopsies and the findings were compatible with those of usual interstitial pneumonia. Immunoelectrophoresis revealed monoclonal gammopathy in both patients. The levels of interleukin 6 in bronchoalveolar lavage fluid were high. In these two patients, idiopathic pulmonary fibrosis was associated with multiple myeloma and monoclonal gammopathy, and the levels of interleukin-6 in bronchoalveolar lavage fluid were high. These findings may help to elucidate the pathogenesis and development of idiopathic pulmonary fibrosis.     

Temporal artery involvement revealing AL amyloidosis and IgD monoclonal gammopathy

We report a patient who presented with clinical symptoms suggesting giant cell arteritis. A diagnosis of AL amyloidosis was made on temporal artery biopsy. Temporal artery involvement is unusual in AL amyloidosis, but its frequency may be underestimated. Another uncommon finding was the presence of an IgD monoclonal gammopathy without diagnostic features of multiple myeloma.     

Monoclonal immunoglobulins

In this review, three topics on monoclonal Ig will be discussed. First, the familial occurrence of Waltenstr?m's macroglobulinemia; second, the biologic features which distinguish benign monoclonal IgM and actual Waldenstr?m's macroglobulinemia; third, the diseases which are strongly associated with monoclonal Ig.     

Peripheral neuropathy and testosterone

A 265 lb. (body mass index, 38.1) male elite bodybuilder suffered focal muscle atrophy that developed in his right vastus lateralis muscle within a month following a painful self-administered injection of testosterone. The subject did not lose any sensory or motor function immediately after the injection; however after several days to weeks, muscle atrophy and paresthesia ensued. The subject had an obvious atrophic zone in the right vastus lateralis muscle with approximately 40% loss of strength when compared to the left leg. In our view, the neuropraxia developed from direct neurotoxic effect of androgens or pressure exerted upon the nerve following injection of a bolus of liquid. This report is indicative of the many potential dangers associated with unsupervised androgen use which may include androgens as potential neurotoxins.     

Diabetic neuropathy

The most common form of diabetic neuropathy is chronic, distal symmetrical sensorimotor, or predominantly sensory neuropathy; the latter is invariably associated with some degree of autonomic dysfunction. There are, however, other neuropathic patterns in diabetes mellitus that are uncommon but are important to recognize, since they may mimic many other non-neurologic diseases. This article discusses a variety of forms of mononeuropathies and diabetic proximal motor neuropathy, commonly known as diabetic amyotropy.     

Diabetic neuropathy

Better clinical characteristics and a standardized approach to the definition of neuropathy has enabled us to define more precisely the natural history of diabetic neuropathy. Detailed studies on the pathology and pathogenesis have allowed dissection of important pathogenetic pathways. Effective treatment is currently limited, although a number of new and potentially important therapeutic interventions, including modification of the vascular supply and antioxidant status and growth factors, may prove to be of benefit in preventing damage and also promoting repair of peripheral nerves in human diabetic neuropathy.     

Unusual case of biclonal gammopathy of undetermined significance]

OBJECTIVE: This was a pilot teleradiology project connecting two secondary KwaZulu-Natal hospitals' radiography departments to a central Durban teaching hospital. The purpose of the study was to assess the usefulness of same-day teleradiology reports to the medical staff and whether such a service changed patient management. DESIGN: After 1 month's service at each hospital, the first 200 teleradiology reports, original radiographs and patients' case notes were reviewed to determine whether any errors in interpretation of the radiographs had been made and whether the reports had changed patient management. RESULTS: The service changed patient management in 10% of cases. Undetected pathology was recognised by the radiologist in 20 patients--pulmonary tuberculosis in 10, spinal tuberculosis in 3, miliary tuberculosis in 2 and fractures in 5. Problems were encountered with transmission of data using the current telephone network, loss of data at the receiving station and the increased workload of the radiographer transmitting the data. CONCLUSIONS: Teleradiology services do make a positive impact on patient management in rural hospitals. However, there are many technical pitfalls that must be avoided in order to establish an effective service.     

Small fiber neuropathy and vasculitis

Vasculitis-associated neuropathy usually presents as multiple mononeuropathies or sensorimotor polyneuropathies that affect large nerve fibers; painful small fiber sensory neuropathy has not previously been described in association with vasculitis. This report describes 2 patients with small fiber neuropathy in whom vasculitis was found to be present. Patients with small fiber neuropathy for which no other cause has been identified should be evaluated for the presence of vasculitis.     

Lyme borreliosis and cranial neuropathy

In a 2-year study of 37 consecutive adult patients with isolated cranial nerve affection of primarily unknown origin, seen at a neurological clinic, borrelia infection was identified as the cause in six cases. Four patients had a peripheral facial palsy and two had a sixth nerve palsy. The patients with borreliosis had headaches or other pain considerably more often than patients with other or unknown aetiology. All six patients had accompanying symptoms and/or signs; in five cases these were obvious, and pointed to a borrelia infection. This study indicates that a careful history to elicit other symptoms of Lyme borreliosis will usually identify the cranial nerve affections with borrelial aetiology in adult patients. To verify the diagnosis, both serum and CSF analysis should be performed. Routine testing for borrelia serology in all patients with cranial neuropathy is generally not indicated.     

Late-onset multiple sclerosis and serum monoclonal gammopathy: an incidental association?

Four patients older than 45 years with a central nervous system demyelinating disease associated to a monoclonal gammopathy are reported. The neurologic disease met the diagnostic criteria of multiple sclerosis, with the particularity of a late onset. Monoclonal paraproteinemia was also present in the cerebrospinal fluid, associated with an intrathecal immunoglobulin synthesis. The clinical data and the course of the disease were comparable to the previous reports of late onset multiple sclerosis. A causal link between the dysglobulinemia and the neurological disease may not be demonstrated. However, such an association may underline the role of humoral processes in multiple sclerosis.