OUTCOMES OF OVERT AND NON-OVERT DISSEMINATED INTRAVASCULAR COAGULATION USING THE ISTH DIC SCORING SYSTEM IN CHILDREN: A SINGLE-CENTER STUDY

Background: Several disseminated intravascular coagulation (DIC) scoring systems are used for prognosticating the clinical outcomes of patients with DIC. However, research on children is scarce. This study compared the clinical outcomes of overt and non-overt DIC using the International Society on Thrombosis and Hemostasis (ISTH) DIC scoring system. Methods: This retrospective study reviewed data on children aged 1 month to 15 years diagnosed with DIC between 2003 and 2014. Results: Of 244 patients, 179 (73.4%) had overt DIC, and 65 (26.6%) had non-overt DIC. The most common causes were infection (84.8%), tissue injury (7%), and malignancies (2.9%). The 28-day case fatality rate was significantly higher for overt than non-overt DIC (76% vs 15.6%; P < 0.001). DIC scores were significantly associated with mortality (R2 = 0.89). Each clinical parameter (platelet count, prothrombin time, and fibrin degradation products) was associated with mortality (P = 0.01). On multivariable analysis, the factors associated with death were platelet counts ≤ 50 000 cells/mm3 (OR, 2.42; 95% CI, 1.08–5.42; P = 0.031); overt DIC score (OR, 7.62; 95% CI, 2.94–19.75; P < 0.001); renal dysfunction (OR, 2.92; 95% CI, 1.34–6.37; P = 0.007); shock (OR, 39.62; 95% CI, 4.99–314.84; P = 0.001); and acute respiratory distress syndrome (OR, 25.90; 95% CI, 3.12–214.80; P = 0.003). Conclusions: The 28-day case-fatality rate was significantly higher for patients with overt than non-overt DIC and concordant with ISTH scores. ISTH DIC scores can be used as a clinical predictor for DIC in children

Isolated Thrombocytopenia in Thai Children: Etiology and Result of Bone Marrow Aspiration Study

Background: Isolated thrombocytopenia (IT) refers to an entity of low platelet count without abnormalities of other lineages. Previous studies revealed that the main cause of IT in children without atypical features i.e. hepa- tosplenomegaly or adenopathy was immune thrombocytopenia (ITP), while acute leukemia and aplastic anemia (AA) were other possible causes. However, there was no previous study in Thailand to identify the cause of IT. Objective: To study the bone marrow aspiration (BMA) result in Thai children with IT and to identify the cause of IT in Thai children. Methods: This was a retrospective chart review of children younger than 15 years old with IT who were diag- nosed at Siriraj Hospital during January 1996 to December 2010. All patients had BMA to identify the cause of thrombocytopenia. Demographic data, clinical manifestation, laboratory results including BMA finding, diagnosis and initial treatment were collected and analyzed. Results: One hundred and twenty-nine patients were enrolled to the study. All patients had normal or increased megakaryocytes in their bone marrow, and none of them had more than 5% of blast cells. Most patients (97.7%) were diagnosed as acute ITP while 2.3% were diagnosed with neonatal alloimmune thrombocytopenia. None of the patients had acute leukemia or AA. Conclusion: The BMA result of most patients in this study was compatible to that of ITP, that is none of the patients had acute leukemia or AA. The most common cause of IT in Thai children is acute ITP. This result sug- gested that BMA in children with IT before starting treatment is not necessary

Mediastinal Germinoma Associated with Hemophagocytic Lymphohistiocytosis: A Case Report

Background: Malignancy-associated hemophagocytic syndrome (MAHS), a secondary form of hemophagocytic lymphohistiocytosis (HLH), can be found with several types of malignancy. It can be manifested either before or after the diagnosis of the underlying malignancy. However, mediastinal germinoma associated HLH has never been reported in previous literatures. Case report: A 13-year-old boy presented with prolonged fever for 10 days with marked hepatosplenomegaly and progressive bicytopenia. Additional investigations demonstrated hyperferritinemia and increased hemophagocytic activity in the bone marrow without evidence of malignancy, compatible with the diagnosis of HLH. He responded well to the HLH-treatment with intravenous immunoglobulins and dexamethasone, but the HLH recrudesced 5 days later. Further investigation revealed anterior mediastinal mass. He quickly deteriorated afterwards and developed pulmonary hemorrhage leading to respiratory failure and died on the following day. Result of the post-mortem tumor biopsy was consistent with mediastinal germinoma. Conclusion: MAHS should be considered in HLH patients who do not respond well or develop recurrence after the appropriate HLH-immunochemotherapy. HLH associated with mediastinal germinoma is rare and fatal. Making diagnosis of the underlying mediastinal germinoma is complicated and challenging. Early diagnosis and prompt treatment of HLH along with the appropriate treatment of germinoma might be the important key for the treatment success

Treatment Outcomes of Kasabach-Merritt Syndrome: A Single Hospital Experience

Objective: Although the outcomes of treatment of Kasabach-Merritt syndrome (KMS) have improved over time, some patients succumb to uncontrollable hemorrhage. Additionally, there is no consensus guideline for the management of KMS. Clinical research on KMS might benefit physicians who treat such patients. Methods: A retrospective chart review of patients diagnosed as KMS by Siriraj Hospital from 2006 to 2016 was conducted. Results: Ten patients were diagnosed with KMS. Four patients underwent surgical intervention and obtained pathological results; 3 of them had kaposiform hemangioendothelioma (KHE), while the fourth had infantile hemangioma (IH). The combination of propranolol and prednisolone, with or without vincristine, was the most common first-line regimen, with a complete response of 37.5%. A combination of vincristine, aspirin, and ticlopidine (VAT) was prescribed as the second-line therapy for 5 patients, but there were no responses in this cohort. Another 2 patients attained hematological remission with embolization and prednisolone monotherapy. A further 2 patients with KHE who were refractory to other treatments responded well to sirolimus, while the tenth patient died of abdominal hemorrhage. Conclusion: The combination of propranolol and prednisolone seems to be effective for KMS. Sirolimus may be considered for salvage therapy for those whose disease is recalcitrant to standard treatment, especially in cases of KMS secondary to KHE. However, research on a larger cohort should be conducted to substantiate the efficacy of such treatments