Treatment of scleromyxoedema with hydroxychloroquine

Background: Scleromyxoedema is a rare disease of unknown aetiology that is characterized by deposition of mucin and sclerotic induration of the skin; it is associated with paraproteinaemia. Patients suffer from progressive disability due to immobilization and cosmetic disfigurement. Treatment of scleromyxoedema is a therapeutic challenge. The antimalarial hydroxychloroquine has a rapid and reliable effect in reticular erythematous mucinosis. Patients and methods: Four consecutive patients (two women, two men; median age: 50 years) with scleromyxoedema, three of them with IgG λ paraprotein, were treated with hydroxychloroquine. Treatment was initiated with 600 mg p. o. for 10 days, followed by 400 mg for at least 4 weeks, and 200 mg thereafter. Results: Complete remission of skin manifestations was achieved in one patient, whereas three patients achieved a partial remission of 61+, 5 and 25 months' duration. Notably, three patients felt increased mobility and reduced firmness of skin during the first week of treatment, which was reflected in a rapid reduction in dermal thickness. In one patient, dysphagia was reverted as evidenced by normalization of oesophageal clearance. Paraproteinaemia was not influenced at all. Side effects included one case of electroretinogram abnormalities after 19 months of therapy and one case of leucopenia after 3 months. Conclusion: Hydroxychloroquine is an effective form of therapy for scleromyxoedema, leading to rapid and prolonged alleviation of symptoms.     

Serum interleukin-6 has no discriminatory role in paraproteinaemia nor a prognostic role in multiple myeloma.

We determined interleukin-6 (IL-6) levels in the serum of 212 well-defined patients with newly diagnosed paraproteinaemia and evaluated its discriminatory value and prognostic role in multiple myeloma (MM). Results were compared with serum neural cell adhesion molecule and beta-2-microglobulin, both established prognostic MM markers. Paraproteinaemia-related diagnoses were: MM (60), other haematological diseases (46), solid tumours (35), autoimmune diseases (17) and monoclonal gammopathy of unknown significance (MGUS) (54). The range of IL-6 levels in all diagnostic groups overlapped widely and did not serve as a discriminatory marker in newly diagnosed paraproteinaemia even when patients with infection or fever (42) were excluded. In MM high IL-6 levels (>/= 50 pg/ml) were not associated with a shorter survival (P = 0.24). We compared our results with 20 published studies on serum IL-6 in paraproteinaemia and/or MM. IL-6 data have to be related to the assay used (bio- or immunoassay) and to the status of MM (newly diagnosed, during therapy, progressive disease). We conclude that serum IL-6 is not specific for paraproteinaemia-related diseases and will not serve as a reliable discriminatory or prognostic marker in paraproteinaemia and MM.     

Pseudotumour Cerebri and Paraproteinaemia

We describe two cases of haematological malignancy, lymphoma and myeloma, presenting with pseudotumour cerebri. A haematological disorder should be considered in the differential diagnosis of the cause of pseudotumour cerebri, and the treatment of pseudotumour cerebri should be considered in the management of haematological disease, especially in avoiding irreversible visual deficit.     

A primary extranodal lymphoma associated with IgM-κ paraproteinaemia: Different secretory capacities of cutaneous and circulating lymphoma cells

We report a patient with non-Hodgkin's lymphoma of small lymphocytic type with IgM-κ monoclonal gammopathy who developed extranodal involvement with orbital and nasal manifestations, followed by generalized subcutaneous nodules. Immunological study disclosed that the peripheral blood and the subcutaneous nodule were both involved in a common monoclonal proliferation of B cells at various stages of differentiation, including secretory cells which accounted for the serum paraprotein. The secretory capacity was far greater in the peripheral blood than in the subcutaneous tissue. These clinical and immunological manifestations might reflect the physiological behaviour of a particular B cell subset which shows a preference for mucocutaneous sites to secrete IgM in the peripheral blood.     

Scleredema associated with paraproteinaemia treated by extracorporeal photopheresis

Scleredema is a rare disease characterized by extensive cutaneous woody, non-pitting induration that spreads throughout the body. We describe a patient with scleredema associated with paraproteinaemia who was successfully treated by extracorporeal photopheresis.     

Enzymological studies in chronic lymphocytic leukemia

Adenosine deaminase (ADA), 5'nucleotidase (5'NT), ecto-5'NT, purine nucleoside phosphorylase (PNP), hypoxanthine-guanine phosphoribosyltransferase (HGPRT), adenine phosphoribosyltransferase (APRT), adenosine kinase (AK), AMP-deaminase (AMPD) and adenylate kinase (AdKin) activities were assayed in peripheral blood lymphoid cells from 20 patients with B-cell type chronic lymphocytic leukemia (CLL). Significantly decreased mean activities of ADA, 5'NT, ecto-5'NT, PNP and AMPD were observed when comparing B-CLL lymphoid cells with control peripheral blood lymphocytes (PBL). AK and AdKin activities however, were found to be higher in B-CLL. Relatively wide ranges of ADA and 5'NT activity were observed. In patients with paraproteinaemia, 5'NT activity was found to be relatively high and in the range of the activities in normal PBL. ADA activity seemed to be slightly higher in patients without paraproteinaemia. No correlation could be found between the enzyme activities and the number of cells rosetting with sheep erythrocytes or bearing surface immunoglobulin (sIg). A relationship was suggested between 5'NT activity and Ig production.     

Cerebral air embolism and the blood-brain barrier in the rat

Cerebral air embolism can have hemodynamic effects such as increases in blood pressure and cerebral blood flow. It has been suggested that these factors play a role for the induction of the blood-brain barrier (BBB) dysfunction. In the present study, 5 microliters air was injected into the right internal carotid artery from a catheter in the external carotid artery after ligation of the extracerebral branches. No consistent change in blood pressure was observed with this small amount of air. Hypercapnia, which increases protein leakage in the brain under conditions of high intraluminal pressure, significantly reduced the extravasation in air embolism. Lidocaine and SITS (4 acetamido-4-isothiocyano-stilbene-2,2-disulfonic acid disodium), two drugs that effectively reduce the albumin leakage in acute hypertension, had no prophylactic effect in cerebral air embolism. Spontaneously hypertensive rats are less vulnerable than normotensive rats to pressure-induced BBB dysfunction but did not significantly differ from controls regarding albumin leakage in the present study. It is concluded that the increased cerebrovascular permeability in air embolism is not related to hemodynamic factors.     

Chronic systemic capillary leak syndrome. Report of a case and review of the literature

Systemic capillary leak syndrome (SCLS) is a rare condition characterized by recurrent episodes of generalized oedema and severe hypotension, associated with paraproteinaemia. In addition to the acute form, a few cases of chronic SCLS have been reported. We describe a 64-year-old woman who was hospitalized because of a 6-month history of progressive generalized oedema with pericardial and pleural effusions, associated with a serum paraprotein. Clinical and laboratory findings were consistent with a chronic form of SCLS. Treatment with prednisone, furosemide and theophylline was started, which led to a gradual improvement in 2 weeks and a persistent remission after 9 months. This report indicates that SCLS may occur in a chronic form, which seems to be responsive to a therapeutic regimen with prednisone, furosemide, and theophylline.     

Hairy Cell Leukaemia Occurring with an Unrelated Paraproteinaemia

A detailed study is described of a case of hairy cell leukaemia, presenting with a serum paraprotein of an immunoglobulin (Ig) class different from that synthesised by the neoplas-tic cells. The case was unusual in its association with leukaemic arthropathy but ultrastruc-turally the hairy cells were typical. By immunofluorenscence and immuno-electron microscopy the neoplastic cells expressed IgAλ both on the cell surface and intracellularly in the rough endoplasmic reticulum, perinuclear space and Golgi apparatus. No Ig was observed in the ribosomal-lamellae complexes. These cells also synthesised and secreted Ig of class Aλ in culture. However the serum paraprotein was of class IgA® and could not be attributed to an abnormal population of plasma cells in the bone marrow. There was no other evidence for myeloma and the IgA® paraproteinaemia appeared to be benign, apparently unrelated to the neoplastic proliferation of hairy cells.