Candida vertebra osteomyelitis in a girl with factor X deficiency.

Candidal vertebra osteomyelitis is a rare condition which occurs primarily in immunocompromised patients. We report a 14-year-old girl with factor X deficiency who developed candida vertebra osteomyelitis during home therapy. The microorganism was probably from a contaminated peripheral cannula used for infusion of factor concentrate. This is the first such case in bleeding disorders to our knowledge.

Augmentation of post transplant immunity: antigen encounter at the time of hematopoietic stem cell transplantation enhances antigen-specific donor T-cell responses in the post transplant repertoire.

After transplant, the immune system is reconstituted by cells derived from both hematopoietic stem cells and peripheral expansion from differentiated donor T cells. After transplant, immune function is poor despite transplantation of mature lymphocytes from immune-competent donors. We tested the hypothesis that early antigen encounter at the time of cell transplant would improve the desired donor T-cell responses. Two independent models of peptide-specific T-cell responses were studied. The model for CD4 cells employed T cells from transgenic (Tg) DO11.11 mice that constitutively express the T-cell receptor for the class II-restricted ovalbumin peptide 323-339. The model for CD8 cells employed non-Tg H2-Db-restricted T-cell responses to the influenza nucleoprotein peptide 366-374. As measured both functionally and by direct imaging of T cells using clonotypic reagents, encounter with specific antigen at the time of T-cell transplantation led to clonal expansion of donor T cells and preservation of donor T-cell function in the post transplant immune environment. Antigen-specific donor T-cell function was poor if antigen encounter was delayed or omitted. Severe parent>F1 graft-versus-host reactions blocked the effect of early antigen exposure. Vaccination of transplant recipients against microbial or leukemia antigens may be worthy of study.

The effect of steroid and intravenous immunoglobulin on thrombopoietin levels in pediatric patients with immune thrombocytopenic purpura.

Thrombopoietin (TPO), which is the main regulator of megakaryo/thrombopoiesis, has been recently cloned and purified, and shown to be useful in discriminating thrombocytopenia due to decreased production or increased platelet destruction. However, there are no detailed investigations about the drug effects on TPO levels during childhood. This study was conducted to measure the TPO levels of children with immune thrombocytopenic purpura (ITP) during steroid and immunoglobulin treatment. Twelve patients with acute ITP were treated with high-dose methyl prednisolone and five patients were treated with intravenous immunoglobulin. Neither steroids nor immunoglobulin were found to have any effect on TPO levels.

Thrombosis during all-trans-retinoic acid therapy in a child with acute promyelocytic leukemia and factor VQ 506 mutation.

Acute promyelocytic leukemia (APL) is often associated with a severe hemostatic disorder, caused by the release of procoagulant and fibrinolytic substances from leukemic blasts. The coagulation profile may exhibit disseminated intravascular coagulation and fibrinolysis or proteolysis. Therefore, heparin and antifibrinolytic agents alone or in combination have been used to prevent severe bleedings. Remission induction with all-trans-retinoic acid (ATRA) is accompanied with rapid correction of hemostatic abnormalities. Thrombosis is a rare complication of APL and may be due to the alterations in hemostasis caused by the disease itself as well as ATRA and antifibrinolytics. Here, the occurrence of thrombosis during induction treatment with ATRA combined with aprotinin and chemotherapy is described in a patient who is homozygous for factor VQ 506 mutation.

Differentiation of benign and malignant cervical lymph nodes: value of B-mode and color Doppler sonography.

OBJECTIVE: To evaluate the efficacy of color Doppler sonography in detecting possible differences in blood flow patterns and B-mode sonographic characteristics between malignant and benign cervical lymph nodes. PATIENTS AND METHODS: During a period of 10 months, the palpable cervical lymph nodes of 39 patients were prospectively evaluated with B-mode and color Doppler sonography. The echogenity, contour, ratio of longitudinal diameter to the transverse diameter and the presence of a hilus were evaluated by B-mode sonography. Capsullary and intranodal arterial flow rates were measured and maximal systolic, end-diastolic, mid-systolic flow velocities with pulsatility, resistivity and acceleration indices were obtained. Final diagnosis was established by excisional biopsy (n:34) and clinical follow-up (n:5). RESULTS: The lymph nodes in 20 cases were malignant and in 19 cases were benign. The B-mode contour characteristics and presence of a hilus in malignant and benign lymph nodes were statistically significant (P < 0.05), but there were no significant differences between the ratio of longitudinal diameter to the transverse diameter (P > 0.05), echogenity (P > 0.05) and the diameters of lymph nodes (P > 0.05). Flow patterns were obtained in 14 malignant (70%) and 10 benign (52.6%) cases. The maximal systolic, end diastolic, mid-systolic flow velocities and pulsatility, resistivity, acceleration indices did not differ statistically (P > 0.05). CONCLUSION: In the differentiations of benign and malignant cervical lymph nodes, the B-mode and color Doppler sonography are limited methods and can not replace biopsy.

Serum fibronectin in neonatal sepsis: is it valuable in early diagnosis and outcome prediction?

The value of the serum fibronectin level in early diagnosis of neonatal sepsis and as a prognostic indicator was investigated. The serum fibronectin levels and Töllner's sepsis scores of 45 neonates who were hospitalized for the suspicion of infection and of 20 healthy neonates as controls were evaluated. Depending on the findings it was concluded that serum fibronectin level varies according to the gestational age, and that the serum fibronectin level is a useful acute phase reactant in the early diagnosis of neonatal sepsis. It can also be used as a prognostic indicator in neonatal sepsis.

Bone marrow hemophagocytosis and immunological abnormalities in a patient with lysinuric protein intolerance.

Lysinuric protein intolerance (LPI) is an inborn error of amino acid transport characterized by a wide spectrum of clinical and biochemical abnormalities. Bone marrow hemophagocytosis in this disorder is an intriguing finding, present mostly in Italian patients. We report a 19-month-old Turkish infant with LPI, bone marrow hemophagocytosis, interstitial lung disease and immunological abnormalities unprecedented in the current literature. Possible etiologic factors responsible for hemophagocytosis and the differential diagnosis of hemophagic syndromes are discussed.