Successful treatment of disseminated BCG infection in a SCID patient with granulocyte colony stimulating factor.

Severe combined immunodeficiencies (SCID) are disorders with impairment of humoral and cellular immune functions. The prognosis of disseminated bacillus Calmette-Guérin (BCG) infection in immunocompromised host is unfavorable since response to standard therapy is poor. We report a successful treatment of disseminated BCG infection with granulocyte colony stimulating factor (G-CSF) in a patient with severe combined immunodeficiency. The patient failed to response to intensive anti-tuberculous (anti-TB) therapy. After 2 months of G-CSF, in addition to anti-TB treatment, the clinical signs of disseminated BCG infection were improved. Since serious BCG infections in SCID are not uncommon in developing countries, where BCG vaccination is mandatory to all newborns, the combination of G-CSF and anti-TB drugs should be considered in immunocompromised patients with protracted mycobacterial infection.

Dengue hemorrhagic fever in patients with thalassemia.

Dengue hemorrhagic fever (DHF) causing by dengue viral infection is endemic in Thailand and Southeast Asian countries where thalassemias are prevalent. Thalassemic patients are also at risk to acquire dengue viral infections and to develop DHF. However, they can have different clinical manifestations and complications as well as more severity than general population requiring special awareness for proper diagnosis and management. We reported 20 thalassemic patients (10 boys and 10 girls) with DHF admitted to Department of Pediatrics, Siriraj Hospital during 1977 to 2001. Their ages ranged from 2-16 years (average 9.5 years). These cases included 5 cases of Hb H disease, 5 cases of Hb H with Hb Constant Spring (CS), 9 cases of beta-thalassemia/Hb E disease and 1 case of beta-thalassemia major. Two cases were in Grade I, 10 cases in grade II, 7 cases in Grade III and one case in grade IV severity of DHF. Though there were evidences of plasma leakage, instead of hemoconcentration, eighteen patients (90 percent) had hematocrit dropped at the range of 11-66% of the initial level. Fifteen patients (75 percent) required at least one packed red cell transfusion. Nine patients (45 percent) had mild bleeding symptoms, one of them had upper gastrointestinal hemorrhage requiring platelet concentrate transfusion. Two patients (10 percent) had serious complications including one with infection-associated hemophagocytic syndrome (IAHS) requiring intravenous immunoglobulin (IVIG) and packed red cell transfusion and the other had generalized seizure due to hyponatremia and hypotension. No mortality was observed among this group of patients. Early recognition of the DHF in thalassemic patients and appropriate packed red cell transfusion in patients with anemic symptoms is warranted to reduce morbidity and mortality in these patients.

Evaluation of guideline for treatment of febrile neutropenia in pediatric cancer at Siriraj Hospital.

BACKGROUND: Febrile neutropenia (FN) is a common and important clinical problem in pediatric cancer. Our Institution has developed a clinical practice guideline (CPG) for treatment of FN to assist the clinicians taking care of these patients.OBJECTIVE: To evaluate characteristics of FN, sources and causative agents of infection, applicability and effectiveness of the CPG, and factors that associated with response to treatment. MATERIALS AND METHODS: The medical records of patients with FN that had completed data from September, 2003 to May, 2005 were reviewed and analysed. RESULTS: A total of 148 FN episodes in 90 patients were analysed. The predominant underlying malignancy was acute leukemia. About 50% had absolute neutrophil count (ANC) less than 100 cells/mm3 at the beginning and at reassesment on day 3 of treatment. The causes of infection with microbiological confirmation was 25%. Urinary tract infection was the predominant source of infection and gram negative bacteria was the predominant causative agent. Sixty-two percents responded to initial treatment without changing of antibiotics. Of all episodes, 91.2% were able to complete treatment according to the CPG. The mortality rate was 1.4%. ANC of less than 100 cell/mm3 on day 3 of treatment was the significant risk factor for prolonged duration of fever and unresponsiveness to low risk regimen of antibiotics. ANC of less than 100 cell/mm3 on day 3, having hematologic malignancies, and recurrent fever were associated risks for the need for antifungal agent or referral to infectious diseases specialist or death. The pretreatment ANC more than 100 cells/mm3 was a significant predictor for the responsiveness to low risk regimen without recurrent fever. CONCLUSION: Our CPG could practically be applied in FN patients and resulted in low mortality rate.

A girl with A glowing tooth : A case of congenital erythropoietic porphyria.

Congenital erythropoietic porphyria is a rare type of porphyria caused by inherited defects of uroporphyrinogen III synthase, an enzyme in the heme biosynthetic pathway. The resultant accumulation of porphyrins causes damage to the skin and erythrocytes, leading to cutaneous photosensitivity and hemolytic anemia. Furthermore, excess porphyrins are also deposited in tissues, bone, and teeth, resulting in a reddish-brown discoloration of the teeth (erythrodontia) which fluoresces under long-wavelength ultraviolet light. In this report, a case of a 9-month old infant girl with recurrent skin eruptions, anemia with hepatosplenomegaly, and erythrodontia is presented. The diagnosis of congenital erythropoietic porphyria was made based on the clinical manifestations and biochemical investigations. The patient was treated successfully with allogenic bone marrow transplantation and is still in remission after almost 3 years posttransplantation.

Thrombotic complications during induction chemotherapy of acute childhood lymphoblastic leukemia.

The incidence of thrombosis during induction chemotherapy of acute childhood lymphoblastic leukemia (ALL) patients was 6 found to be in 105 (5.7%). There were 4 cerebral infarctions, 1 superior vena cava (SVC) obstruction and 1 deep vein thrombosis. Among these, 2 of them died. A prospective study was further conducted of the change in coagulation and anticoagulation factors during 6 weeks of induction chemotherapy. It was found that the activated partial thromboplastin time (aPTT) was within normal range in all cases throughout 6 weeks, while prothrombin time (PT) and thrombin time (TT) were slightly prolonged, especially during the first 3 weeks of this phase. The natural anticoagulant panels which included protein C (PC), protein S (PS) and antithrombin III (AT III) and also fibrinogen level, were lower during the first 3 weeks and reached its nadir during the second and third week. The lower level of natural anticoagulants might be an important predisposing factor for the occurrence of thrombosis in these patients.

Malignancies in HIV-infected children at Siriraj Hospital.

BACKGROUND: Some malignancies such as Kaposi's sarcoma, non-Hodgkin's lymphoma (NHL) are one of the acquired immunodeficiency syndrome (AIDS)-defining illnesses. With the improving survival of patients with AIDS due to better prevention and treatment of infectious complications, there may well be an increase in AIDS-related malignancies. OBJECTIVE: To study malignancies in human immunodeficiency virus (HIV)-infected children in view of demographic data, HIV disease status, characters of malignancies, and treatment outcome. METHOD: Retrospective study was performed in HIV-infected children with malignancies at Siriraj Hospital from January 1995 to October 2001. RESULTS: During the 6 year and 10 month period, there were 7 HIV-infected children (2 boys, 5 girls) with malignancies. Mean age at diagnosis of malignancies was 3 years 7 months (2 years 6 months-5 years). Hepatomegaly and lymphadenopathy were the most common presenting symptoms. All patients had NHL stage III or IV. Burkitt's lymphoma was the predominant type. Six patients were treated with appropriate chemotherapy and one patient also received antiretroviral therapy. Only one patient with large cell lymphoma stage IV who received both antiretroviral and chemotherapy has survived to date. Five patients died during chemotherapy treatment and one patient died before receiving chemotherapy. Causes of death of these patients were infections. One of them with Burkitt's lymphoma stage III also had central nervous system (CNS) relapse at the time of death. Mean survival time after diagnosis with malignancies was 11 months (15 days-3 years 1 month). CONCLUSION: NHL is the most common malignancy in HIV-infected children at Siriraj Hospital. Age at presentation of NHL in these children is younger than their non-HIV counterpart. Outcome of treatment is poor. Adjustment protocol for treatment of malignancy in HIV-infected children combined with antiretroviral therapy for controlling HIV infection should be studied further.

Secondary hemophagocytic lymphohistiocytosis in children: an analysis of etiology and outcome.

Fifty-two pediatric patients were diagnosed with secondary hemophagocytic lymphohistiocytosis (HLH) at the Department of Pediatrics, Siriraj Hospital between 1989 and 1998. Of these, 15 were infection-associated (IAHS), 25 were malignancy-associated (MAHS) and 12 were idiopathic HLH. Causative organisms for IAHS were Salmonella (3), Staphylococcus (2), enterobactor (2), dengue virus (3), malaria (2) and one each of Ebstein Barr virus (EBV), Serratia marcesens and Penicillium maneffei. Unlike those reported in adults and in the Western literature, 47 of 52 children in the present series were immunocompetent hosts. In addition, the proportion of MAHS was higher than expected (48.1%). Twenty-two of 25 MAHS presented with hemophagocytic syndrome and were subsequently found to have malignant diseases. Sixty per cent of MAHS (15 cases) were associated with non-Hodgkin's lymphoma (NHL), mainly T-cell. Other malignancies included acute leukemias (7) MDS (1), Langerhans cell histiocytosis (1) and histiocytic sarcoma (1). Treatment approaches were specific therapy for individuals with known causes. Supportive treatment with blood components transfusions, steroid, intravenous immunoglobulins (IVIG), and chemotherapeutic agents, mainly vinblastine and etoposides, were used in indicated cases. Of the 52 cases, 15 (28.8%) had a fatal outcome during the acute phase, and other 4 died of their subsequent malignant diseases. There was a statistically significant association between poorer prognosis and patients' age < 3 years (p= 0.004) or MAHS (p=0.005). Conclusion: Secondary HLH is not uncommon in Thai children who are immunocompetent. Malignancies, particulary NHL, are highly suspicious especially for cases not responsive to conventional therapy. Poor prognostic factors are age less than 3 years and MAHS.