Thrombocytopenia in neonates born to women with autoimmune thrombocytopenic purpura.

We conducted a survey by questionnaire to clarify the actual conditions of neonates born to mothers with autoimmune thrombocytopenic purpura (ATP) in Japan. We investigated 93 pregnancies (1 resulting in twins) in 31 hospitals between 1985 and 1994. Forty-nine of the neonates (52%) had thrombocytopenia (below 150 x 10(9)/L). Nineteen neonates (20%) showed a bleeding tendency, but this was generally mild. In only one neonate (1%) (a case of asymptomatic intracranial hemorrhage, ICH), deep bleeding occurred due to thrombocytopenia. The lowest platelet count of neonates after birth occurred on day 4, not on day 0. There was no correlation between maternal and neonatal platelet counts. However, there was an apparent correlation between the neonatal platelet count on day 0 and the lowest platelet count after birth. Treatment of the mothers with intravenous high-dose gamma-globulin and prednisolone did not prevent risk of neonatal thrombocytopenia significantly.

Three cases of hereditary nonspherocytic hemolytic anemia associated with red blood cell glutathione deficiency.

Three unrelated Japanese patients with chronic nonspherocytic hemolytic anemia wer found to have marked deficiency of red blood cell (RBC) reduced glutathoine (GSH) (4.4%, 13.1%, and 6.9% of normal, respectively). A panel of RBC enzyme assays showed that one patient had decreased glutathione synthetase activity and the other two were moderately deficient in gamma-glutamylcystine synthetase. Some family members of each patient showed mild deficiency of the respective enzymes. RBCs of these patients also showed a decreased level of glutathione-S-transferase as in previously described GSH-deficient cases. Hemolytic anemia was their only manifestation, and neither 5-oxoprolinemia nor 5-oxoprolinuria, which are usually associated with to generalized type of glutathione synthetase deficiency, was noted in our patients.

Autoantibodies and CD5+ B cells in childhood onset immune thrombocytopenic purpura.

We evaluated platelet associated immunoglobulin (PIag) G, PaIgM, platelet associated autoantibodies to platelet glycoprotein IIb/IIIa (Pa-GPIIb/IIIa), the percentage of CD5+ B cells and the amount of platelet-bound anti-GPIIb/IIIa monoclonal antibody (mAb) in the peripheral blood of 29 patients with childhood onset chronic immune thrombocytopenic purpura (c-ITP). The percentage of CD5+ B cells ranged from 2 to 8% (4.7 +/- 2.0) in control patients and 1 to 18% (6.2 +/- 4.2) in the ITP patients. There was no overall significant difference between the two groups, but the percentage of CD5+B cells in six of the ITP patients was higher than the mean + 2 s.d. of the controls. There was a significant correlation between the percentage of CD5+ B cells and PaIgM (y = 1.73x + 13.4, r=0.40, P < 0.05). This finding is the basis for the speculation that CD5+ B cells may play an important role in the production of PaIgM in vivo. There was no correlation between the amounts of PaIgG and Pa-GPIIb/IIIa). This suggests that the amount of PaIgG does not accurately reflect of the amount of Pa-GPIIb/IIIa. Furthermore, we have demonstrated that autoantibodies to GPIIb/IIIa are directed to more that one epitope.

[A case report of Epstein syndrome].

Epstein's triad which is a syndrome with the combination of macrothrombocytopenia, deafness and nephritis, is similar to Alport's syndrome. We report on a case of Epstein syndrome and describe the results of morphological examination of a renal biopsy, specimen. The patient was a 14-year-old girl with the diagnosis of chronic idiopathic thrombocytopenic purpura that had preseated from the age of 3 years. She was referred to Daisan Hospital of the Jikei University School of Medicine on April 1, 1991 for refractory thrombocytopenia. She had shown sensorineural hearing loss since the age of 6 years and her peripheral blood smear revealed giant platelets on admission. She was treated with interferon, prednisolone, and high-dose gamma-globulin (400 mg/day x 5 days). However, the platelet count did not increase, but hypermenorrhea continued. She subsequently showed proteinuria and hematuria. She underwent splenectomy and renal biopsy on August 12, 1992. The glomeruli appeared to be almost normal under light microscopy. The interstitium showed regional fibrosis containing foam cells and the renal tubuli showed mild atrophy. Under electronmicroscopy, the basement membrane of the glomeruli was associated with mesangial interposition and the lamina densa was split into several layers. These ultrastructural findings were compatible with those of Alport's syndrome.

Lymphoma syndrome leukemia revealed interesting finding on brain CT and ultrasound scan of abdomen at the initial presentation.

This paper reports on a patient with lymphoma syndrome leukemia (LSL) who showed interesting findings on brain computed tomography (CT) and ultrasound scans of the abdomen at the initial presentation. The patient was a 5 year old girl. When she was admitted to our hospital, there were many lymph nodes palpable. The abdomen was distended and the liver and spleen were palpable below the umbilicus. Hematologic examinations revealed a leukocyte count of 275,800/microL with 98% lymphoblasts. Chest X-ray film revealed a mediastinal mass. The diagnosis of LSL was made. Her brain CT scan showed a low density area in the right thalamic region without contrast enhancement; infarction was suspected. Furthermore, her abdominal ultrasound scan showed hepatosplenomegaly, kidney swelling with increasing echogenicity and hydronephrosis and stones in the renal pelvis and bladder. These findings are unprecedentedly rare in cases of childhood acute lymphoblastic leukemia (ALL), much less in LSL.

Establishment and characteristics of a T-cell acute lymphoblastic leukemia cell line, JK-T1, with a chromosomal translocation between 8q24 and 14q13.

A human leukemia cell line, JK-T1, was established from the bone marrow of a 10-year-old boy with T-cell acute lymphoblastic leukemia. The origin of the leukemic cell line, JK-T1, was demonstrated by its chromosomal and immunologic similarity to the patient's fresh leukemic cells. Karyotypic analysis revealed 46,XY,del(6)(q?),t(8;14)(q24;q13),der(9)t(9;?)(q34;?). In JK-T1, neither rearrangement nor amplification of the c-myc gene was observed apparently because the breakpoint of chromosome 14 was not q11 but q13. JK-T1 was independent of interleukin 2 (IL-2) because of little production of IL-2, little IL-2 receptor (CD25) on the surface, and no response to exogenous IL-2. JK-T1 had lymphocyte function associated antigen-1 (LFA-1) (CD11a, CD18) on its surface and could adhere to the hematologic stromal layer. These characteristics of JK-T1 cell line are considered to be useful not only for evaluating the role of t(8;14) but also in studying the adhesion molecules of leukemia.

Summer-type hypersensitivity pneumonitis in a child.

Summer-type hypersensitivity pneumonitis (HP) is a unique disease in Japan. The clinical features of this disease are as follows: 1) cough, fever and dyspnea as a clinical triad, 2) diffuse reticulonodular opacities on the chest X-ray film, 3) restrictive impairment and decrease in DLco, 4) hypoxia, 5) initiation in summer, 6) worsening of the condition when the patient returns home, 7) granuloma formation and alveolitis in the lung biopsy specimen, 8) familial clustering. The etiologic agent of this disease is debatable. In 1984 Ando et al reported that the etiologic agent was T. cutaneum. Now many people are pursuing the argument to its logical conclusion. We report a case of summer-type HP. It is uncommon in children, especially in a child in whose serum antibody to T. cutaneum can be demonstrated.