HTLV-1-associated myelopathy/tropical spastic paraparesis with pseudohypoparathyroidism.

In many short-stature patients with human T-lymphotrophic virus type I-associated myelopathy/tropical spastic paraparesis (HAM/TSP), signs and symptoms were manifested during childhood. Successive investigations revealed 12 of 14 short-stature patients with pseudohypoparathyroidism (PHP) from the findings of short metacarpi, parathyroid hormone infusion test, immunoblotting of erythrocyte membrane, or lymphocytic Northern blotting of Gsalpha. Patients with PHP probably showed HAM/TSP based on their modified immunologic status. Human T-lymphotrophic virus type I infection did not induce PHP, but PHP may be a risk factor for the occurrence of HAM/TSP.

Adenosine and neopterin levels in cerebrospinal fluid of patients with neurological disorders.

We determined the cerebrospinal fluid (CSF) levels of adenosine, a mediator of cerebral blood flow regulation, and neopterin, a macrophage-producing compound, in patients with neurological disorders. Compared to control subjects, the adenosine levels were significantly increased in the patients with acute-stage cerebral infarction (n=12, p<0.0001), acute meningitis (n=10, p<0.0001), or amyotrophic lateral sclerosis (ALS, n=12, p<0.05) (Mann-Whitney U-test). The neopterin levels were significantly increased in the 41 patients with human T-lymphotropic virus type I-associated myelopathy/tropical spastic paraparesis (HAM/TSP, p<0.0001), acute meningitis (p<0.0001), ALS (p<0.05) (Mann-Whitney U-test), or acute-stage cerebral infarction (p<0.005, Student's t-test). In the analysis of 41 HAM/TSP patients, the neopterin levels were significantly correlated with the cell number and glucose levels in the CSF, and were a sensitive marker of inflammation. Several of the HAM/TSP patients with increased adenosine levels were probably complicated with other diseases. The increased neopterin levels in the HAM/TSP group persisted, suggesting that the mononuclear cellular infiltration remained for a long time.

[Markedly elevated serum neopterin levels in patients with hemophagocytic histiocytosis].

The authors measured serum neopterin by HPLC in 3 patients with hemophagocytic histiocytosis (HH) with DIC which occurred during the clinical course of T cell malignant lymphoma (T-ML). Extremely high levels of serum neopterin (754.3 +/- SD 467.3 pmol/ml) were found in the patients, about 200, 13 and 10 times higher than that of normal subjects, non-HH DIC patients and non-HH/DIC T-ML patients. Moreover their serum soluble interleukin-2 receptor levels were markedly elevated. These results indicate that markedly raised serum neopterin would be a sensitive parameter for activation of the mononuclear phagocyte system closely associated with T-cell activation and may serve as a useful diagnostic marker for HH.

[Plasma concentrations of plasminogen activator inhibitor 2 in patients with hematological malignancies and their clinical significance].

We investigated plasma levels of plasminogen activator inhibitor 2 (PAI-2) in 40 patients with hematological malignancies including acute leukemia, chronic leukemia, hemophagocytic histiocytosis (HH) and histiocytic sarcoma (HS). The plasma PAI-2 levels in the patients with mononuclear phagocyte system (MPS) proliferative disorders (M4, M5, and CMMoL on FAB classification, HH and HS) were all above the cut-off value in healthy subjects. In a patient of reactive HH, there was a close correlation between Plasma PAI-2 and serum ferritin levels during the clinical course. These results indicate that the increase of plasma PAI-2 would be a diagnostic indicator of MPS proliferative disorders.