Angiocentric T-cell lymphoma of the pancreas presenting as late-onset diabetes mellitus with diabetic retinopathy.

Pancreatic lymphoma presenting with clinical diabetes mellitus (DM) is rare. We report angiocentric T-cell lymphoma of the pancreas in a 65-year-old Thai woman who presented with progressive deterioration of visual acuity of both eyes. She had diabetic retinopathy (DR) diagnosed only 20 months after the diagnosis of DM at the age of 63. She later developed erythematous rashes, fever, and deterioration of consciousness; she eventually died of shock. A skin biopsy and bone marrow examination revealed angiocentric T-cell lymphoma. At autopsy, the pancreas and both eyes were extensively infiltrated by lymphoma. Widespread involvement of nearly all organs but superficial lymphadenopathy was detected. In contrast to other typical cases of long-standing DM, only mild atherosclerosis was noted, and no DR was found. To the best of our knowledge, this is the first case of lymphoma involving the pancreas and both eyes producing clinical DM and DR.

Detection of molecular variants of BCR-ABL gene in bone marrow and blood of patients with chronic myeloid leukemia by reverse-transcriptase polymerase chain reaction (RT-PCR).

Very limited data exists in Thailand regarding the frequency of BCR-ABL leukemic gene and its prognostic implication in Thai CML patients. The objective of this study was to develop a rapid molecular assay for the detection of the two most commonly reported variants of BCR-ABL fusion gene, B2A2 and B3A2 in CML patients. Bone marrow or peripheral blood were used for RNA extraction and reverse-transcribed to cDNA for PCR amplification. 92 per cent of CML patients (91/99) were positive for BCR-ABL gene (61% B3A2 and 31% B2A2). 8/99 CML patients were BCR-ABL-negative. B3A2 and B2A2-positive patients did not have any different clinical and hematological features at presentation although B3A2 patients tended to be slightly older and had higher platelet counts. 71/71 non-CML including other MPD and leukemia cases were all negative for BCR-ABL gene. In conclusion, a rapid RT-PCR assay has now been developed for the detection of this hallmark gene in CML patients. It should be of great value in the differential diagnosis of CML from other diseases. Long-term follow-ups of CML patients with different variants are needed to determine the prognostic importance of each gene variant.

Serum transcobalamin II level in glucose-6-phosphate dehydrogenase deficient subjects with typhoid fever.

Transcobalamin II (TCII) is the vitamin B12 binding protein which is responsible for delivery of this vitamin to the tissues. High values for serum TCII have been reported in many clinical conditions. This paper describes the elevated serum TCII levels in three G-6-PD deficient patients with typhoid fever. They had severe hemolysis with hemoglobinuria associated with slight liver dysfunctions but without obvious increased serum creatinine and BUN concentrations. A remarkable increase in serum TCII level was observed during active hemolysis and decreased to the normal level within 2-3 days after hemolysis ceased. The mechanism of increased serum TCII during hemolysis is probably due to hemoglobinuria secondary to excessive hemolysis. As Hb is known to be efficiently reabsorbed by the proximal tubule cells and can competitively inhibit the tubular uptake of TCII-B12. It is possible that excess Hb interferes with TCII uptake and degradation at renal tubular cells. Therefore, the circulating TCII survival is prolonged resulting in the elevated TCII level. Furthermore, lysosomal degradation of newly synthesized TCII is a normal process that regulates the TCII secretion. Therefore, a reduced lysosome-mediated uptake of TCII-B12 by renal tubular cell may stimulate the TCII secretion as has been shown experimentally in vitro.

Therapy of chronic idiopathic thrombocytopenic purpura in adults: experiences from Thailand.

Results of different types of treatment in 416 adults with chronic idiopathic thrombocytopenic purpura (ITP) were analyzed. Of 368 patients treated with corticosteroids, 278 (77%) achieved complete response (CR ie, normalization of platelet count). However, only 18% of patients had continued complete response (CCR). Results of splenectomy was analyzed in 126 patients, 49% of them achieved CCR and 76% of them had beneficial effects from the operation. Emergency splenectomy was a life saving procedure in 8 of 11 patients with life-threatening hemorrhage. Vinca alkaloids gave a 50% transient CR. Immunosuppressive agents (cyclophosphamide, azathioprine) resulted in only 11% CCR.

Serious complications following treatment of chronic idiopathic thrombocytopenic purpura.

Six patients had serious complications as consequences of treatment of idiopathic thrombocytopenic purpura. Five had splenectomy-related complications, one of them developed fatal intra-abdominal bleeding. Three patients acquired operation-related serious infection, two of them died. Serious neutropenia after vinblastine-loaded platelets occurred in one patient leading to pseudomonas septicaemia and panophthalmitis with permanent vision loss of left eye. Recurrence thrombocytopenia occurred in every case during serious complications. Early detection by awareness of the possibility of serious complications can reduce morbidity and mortality occurring after therapy of idiopathic thrombocytopenic purpura.

A WHO collaborative study on iron supplementation in Burma and in Thailand.

Studies on the treatment and prevention of iron deficiency anemia, in pregnant and nonpregnant women and in men, were conducted in Thailand and Burma. The effects of the dose of Fe, duration of Fe administration, additional supplementation with folate, mode of supplement delivery (either supervised or unsupervised), and the presence of Hb(AE) were studied. The frequency and severity of side effects were also recorded. Fe administration resulted in an increase in hemoglobin concentration in all anemic individuals but approximately 20% failed to reach normality. The length of administration and the dose influenced the results. Frequency and severity of side effects increased with the dose of Fe administered. Folate supplementation did not affect the results. It appears possible to integrate a program of prevention and treatment of Fe deficiency anemia in a primary health-care system but the constraints and limitations of achievable results should be recognized.

PIP: 4 collaborative trials of iron supplements with or without folate on the following groups: adult men and women in central Thailand; pregnant women in northeastern Thailand; adult women in northern Thailand; and pregnant women in Burma, are reported as separate studies here. Subjects took 30, 60, 120 or 240 mg Fe sulfate with or without 2.5 mg folic acid. Those with chronic disease or hemoglobin 80 g/L were excluded. Presence of hemoglobin EE or EF, or hookworm infestations were determined in some cases. Hemoglobin E, which tends to increase incidence of anemia, occurs in up to 4.8% of women in northern Thailand, 27.6% of adults in northeastern Thailand, and 19% of pregnant women in Burma. Hookworm infection was a high as 35% in central Thais. Iron supplementation significantly increased mean Hb and reduced incidence of anemia in all study groups. 20% of subjects remained anemic. In northeastern Thailand treatment for 15 weeks resulted in higher Hb than did 10 weeks treatment. No significant difference was seen with dose of Fe or inclusion of folate. Supervised administration of supplements had no additional effect. Gastrointestinal side effects were a problem, and were dose related, but tended to resolve after a while. The fact that Hb levels did not rise to normal levels indicates that a combined program possibly including parasite control and food fortification should be considered.

Iron absorption in patients with beta-thalassaemia/haemoglobin E disease and the effect of splenectomy.

Intestinal 59Fe absorption was measured in 16 patients with beta-thalassaemia/haemoglobin E disease and in 5 normal controls, using a total-body counting technique. The average iron absorption in the patients was 62% in contrast to 16.5% in the normal controls. 6 of the 9 splenectomized patients had absorption values above 65%, while only 2 of the 7 nonsplenectomized patients had comparably high values.

Zinc and copper status in hemoglobin H disease and beta-thalassemia/hemoglobin E disease.

The plasma zinc and copper and urinary zinc levels were determined by an atomic absorption spectrophotometer technic in 14 patients with hemoglobin (Hb) H disease, 34 patients with beta-thalassemia/Hb E disease and 30 control subjects. Plasma zinc levels were lower and the plasma copper levels were higher in the patients than in controls. The plasma Cu:Zn ratios, a more valuable indicator of body zinc status, increased in the diseased subjects, indicating zinc deficiency. Higher urinary zinc levels in the both Hb H disease and 30 control subjects. Plasma zinc levels were lower and the plasma copper levels were higher in the patients than in controls. The plasma Cu:Zn ratios, a more valuable indicator of body zinc status, increased in the diseased subjects, indicating zinc deficiency. Higher urinary zinc levels in the both Hb H disease and 30 control subjects. Plasma zinc levels were lower and the plasma copper levels were higher in the patients than in controls. The plasma Cu:Zn ratios, a more valuable indicator of body zinc status, increased in the diseased subjects, indicating zinc deficiency. Higher urinary zinc levels in the both Hb H disease and beta-thalassemia/Hb E disease suggest that chronic hemolysis is responsible for hyperzincuria leading to zinc deficiency. The thalassemic patients with undergrowth had higher plasma Cu:Zn ratios than the patients with normal development, indicating a more severe zinc deficiency in the former.

Serum erythrocyte folate levels in thalassaemic patients in Thailand.

The mean serum and adjusted red cell folate levels, as measured by microassay using Lactobacillus casei, respectively were 8.87 +/- SD 3.28 microgram/l and 436 +/- SD 107 microgram/l in 76 normal subjects, 4.22 +/- SD 2.70 microgram/l and 182 +/- SD 114 microgram/l in 55 patients with beta-thalassaemia/Hb E disease and 6.36 +/- SD 2.95 microgram/l and 320 +/- 158 microgram/l in 37 patients with Hb H disease. The mean serum and adjusted red cell folate values of the patients with beta-thalassaemia/Hb E disease and the mean serum folate value of the patients with Hb H disease were significantly lower than those of the normal subjects (P less than 0.001). 33% of the beta-thalassaemia/Hb E patients and 8% of the Hb H patients showed low serum folate levels (less than 3 microgram/l) whereas 84% of the former and 45% of the latter showed low adjusted red cell folate levels (less than 270 microgram/l). The group of beta-thalassaemia/Hb E disease with low serum folate levels had lower mean haemoglobin concentration and lower mean adjusted red cell folate level than the group with normal serum folate levels. Since the Thai diets have high folate content, the observed low serum and erythrocyte folate levels in thalassaemic patients most likely occur from massively increased erythropoiesis. Folate, 5 mg/d, is now routinely prescribed to such patients especially to those with severe anaemia.