A two-month-old boy visited the hospital due to unexpected cutaneous purpura and thrombocytopenia for 2 days. The physical examination revealed a purple mass on the back. The soft tissue color Doppler ultrasound showed rich blood signals in the tissue, and the results of bone marrow puncture indicated an increased number of megakaryocytes. After the treatment with hormone and gamma globulin, the platelet count rapidly increased and maintained at a normal level. Meanwhile, the boy was given oral administration of propranolol. He was followed up for 4 months and the volume of the mass on the back was reduced significantly. He had a definite diagnosis of hemangioma and immune thrombocytopenia. As for the patients with hemangioma complicated by thrombocytopenia, knowledge of Kasabach-Merritt syndrome should be enhanced and there should be a clarification of the association between thrombocytopenia and hemangioma. There should also be an alertness for thrombocytopenia of other causes.
Humans ; Infant ; Male ; Platelet Count ; Purpura ; blood ; drug therapy ; etiology ; Thrombocytopenia ; etiology

Previous studies reported that oxaliplatin is associated with sinusoidal obstruction syndrome. However few reports on oxaliplatin induced liver fibrosis are found in the literature. Furthermore pathogenesis of liver fibrosis is not well known. We report a case of 45-yr-old Korean man in whom liver fibrosis with splenomegaly developed after 12 cycles of oxaliplatin based adjuvant chemotherapy for colon cancer (T4N2M0). Thorough history taking and serological examination revealed no evidence of chronic liver disease. Restaging CT scans demonstrated a good response to chemotherapy. Five month after chemotherapy, he underwent right hepatectomy due to isolated metastatic lesion. The liver parenchyma showed diffuse sinusoidal dilatation and centrilobular vein fibrosis with necrosis without steatosis. We could conclude that splenomegaly was due to perisinusoidal liver fibrosis and liver cell necrosis induced portal hypertension by oxaliplatin. In addition, to investigate the pathogenesis of liver fibrosis, immunohistochemical stains such as CD31 and alpha-smooth muscle actin (alpha-SMA) were conducted with control group. The immunohistochemical stains for CD31 and alpha-SMA were positive along the sinusoidal space in the patient, while negative in the control group. Chemotherapy with oxaliplatin induces liver fibrosis which should be kept in mind as a serious complication.
Actins/metabolism ; Antigens, CD31/metabolism ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; Camptothecin/*analogs & derivatives/therapeutic use ; Chemotherapy, Adjuvant ; Colonic Neoplasms/*drug therapy ; Fluorouracil/therapeutic use ; Humans ; Hypertension, Portal/etiology ; Immunohistochemistry ; Leucovorin/therapeutic use ; Liver Cirrhosis/*diagnosis/etiology/pathology ; Liver Neoplasms/secondary/surgery ; Male ; Middle Aged ; Organoplatinum Compounds/*administration & dosage/adverse effects/therapeutic use ; Splenomegaly/*diagnosis/etiology ; Thrombocytopenia/etiology ; Tomography, X-Ray Computed

BACKGROUND/AIMS: Hepatic arterial infusion chemotherapy (HAIC) with 5-fluorouracil and cisplatin for intractable advanced hepatocellular carcinoma (HCC) may have survival benefits. We aimed to determine the efficacy and safety of HAIC for advanced HCC as first-line therapy. METHODS: A total of 54 patients who received only HAIC with 5-fluorouracil (750 mg/m2 on days 1-4) and cisplatin (25 mg/m2 on days 1-4) for advanced HCC from Jan. 2009 to Dec. 2011 were selected. According to Child-Pugh class, the overall survival (OS), progression-free survival (PFS), and adverse events after HAIC were investigated retrospectively. RESULTS: Median OS and PFS between the Child-Pugh A group (n=24) and the Child-Pugh B/C group (n=30) were 8.7 (95% confidence interval [CI]: 4.7-12.7) vs. 3.7 months (95% CI: 2.0-5.3), and 7.1 (95% CI: 3.8-10.4) vs. 3.6 months (95% CI: 2.0-5.2), respectively. Although median OS and PFS were not statistically significant between the two groups (P=0.079, P=0.196), the Child-Pugh class B/C tended to influence poor OS. Serious adverse events > or = grade 3 occurred frequently in both groups (83.3 vs. 96.7%, P=0.159). Responders (22.2%, complete or partial response) significantly differed in median OS, compared to non-responders (13.1 vs. 4.4 months, P=0.019). Achievement of complete or partial response was an independent prognostic factor of OS (hazard ratio: 0.4, 95% CI: 0.2-0.8, P=0.011). CONCLUSIONS: Achievement of response after HAIC provide a survival benefit in patients with advanced HCC, but HAIC should be administered cautiously in patients with Child-Pugh class B/C, because of a relatively low survival and high incidence of serious adverse events.
Adult ; Aged ; Anemia/etiology ; Antineoplastic Agents/adverse effects/*therapeutic use ; Carcinoma, Hepatocellular/*drug therapy ; Cisplatin/adverse effects/*therapeutic use ; Diarrhea/etiology ; Disease-Free Survival ; Drug Therapy, Combination ; Female ; Fluorouracil/adverse effects/*therapeutic use ; Humans ; Infusions, Intra-Arterial ; Kaplan-Meier Estimate ; Liver Neoplasms/*drug therapy ; Male ; Middle Aged ; Neutropenia/etiology ; Retrospective Studies ; Severity of Illness Index ; Thrombocytopenia/etiology ; Treatment Outcome

Treatment of thrombocytopenia in systemic lupus erythematosus (SLE) is considered in cases of current bleeding, severe bruising, or a platelet count below 50,000/microliter. Corticosteroid is the first choice of medication for inducing remission, and immunosuppressive agents can be added when thrombocytopenia is refractory to corticosteroid or recurs despite it. We presented two SLE patients with thrombocytopenia who successfully induced remission after intravenous administration of low-dose cyclophosphamide (CYC) (500 mg fixed dose, biweekly for 3 months), followed by azathioprine (AZA) or mycophenolate mofetil (MMF). Both patients developed severe thrombocytopenia in SLE that did not respond to pulsed methylprednisolone therapy, and started the intravenous low-dose CYC therapy. In case 1, the platelet count increased to 50,000/microliter after the first CYC infusion, and remission was maintained with low dose prednisolone and AZA. The case 2 achieved remission after three cycles of CYC, and the remission continued with low dose prednisolone and MMF.
Azathioprine/therapeutic use ; Bone Marrow/pathology ; Cyclophosphamide/*therapeutic use ; Drug Therapy, Combination ; Female ; Humans ; Immunosuppressive Agents/*therapeutic use ; Infusions, Intravenous ; Lupus Erythematosus, Systemic/complications/*diagnosis ; Middle Aged ; Mycophenolic Acid/analogs & derivatives/therapeutic use ; Platelet Count ; Thrombocytopenia/*diagnosis/*drug therapy/etiology ; Young Adult

The aim of this study was to assess the clinical efficacy and safety of chelation treatment with penicillamine (PCA) in cross combination with sodium 2, 3-dimercapto-1-propane sulfonate (DMPS) repeatedly in patients with Wilson's disease (WD). Thirty-five patients with WD were enrolled. They were administrated intravenous DMPS in cross combination with oral PCA alternately which was practiced repeatedly, all with Zinc in the meantime. During the treatment, clinical observations and 24-h urine copper excretion as well as adverse effects of medicines were recorded and analyzed. Although the incidence of adverse effects was not significantly different after either intravenous DMPS or oral PCA treatment, levels of 24-h urine copper tended to be higher after short-term intravenous DMPS than that of oral PCA. Adverse effects in the course of intravenous DMPS were mainly neutropenia, thrombocytopenia, allergic reaction and bleeding tendency. As compared with oral PCA alone or intravenous DMPS alone, such repeated cross combination treatment could as much as possible avoid continued drug adverse effects or poor curative effect and had less chance to stop treatment in WD patients. Improved or recovered liver function in 71% of the patients, alleviated neurologic symptoms in 50% of the patients, and disappeared hematuria in 70% of the patients could be observed during the follow-up period of 6 months to 5 years after such combined chelation regimen. Chelation treatment repeatedly with oral penicillamine in cross combination with intravenous DMPS alternately could be more beneficial for WD patients to relieve symptoms, avoid continued drug adverse effects and maintain lifelong therapy.
Administration, Oral ; Adolescent ; Chelating Agents ; administration & dosage ; adverse effects ; therapeutic use ; Chelation Therapy ; adverse effects ; methods ; Child ; Copper ; urine ; Drug Administration Schedule ; Drug Hypersensitivity ; etiology ; Drug Therapy, Combination ; Hepatolenticular Degeneration ; drug therapy ; Humans ; Injections, Intravenous ; Male ; Neutropenia ; chemically induced ; Partial Thromboplastin Time ; Penicillamine ; administration & dosage ; adverse effects ; therapeutic use ; Prothrombin Time ; Thrombocytopenia ; chemically induced ; Time Factors ; Treatment Outcome ; Unithiol ; administration & dosage ; adverse effects ; therapeutic use

Blood Platelets ; physiology ; Cytokines ; blood ; Drug Therapy, Combination ; Humans ; Immune Tolerance ; Thrombocytopenia ; drug therapy ; etiology ; immunology

Anaphylactic transfusion reactions are rare complications of blood transfusions. Anhaptoglobinemia, a condition that has high incidence in Asia, can cause allergic transfusion reactions or anaphylaxis in severe cases. A 50-yr-old Korean woman was diagnosed with relapsed acute promyelocytic leukemia. She developed thrombocytopenia during chemotherapy and an anaphylactic transfusion reaction on the 4th and 5th platelet transfusions immediately after the transfusion of the platelet concentrates was initiated. Blood analysis showed no detectable serum haptoglobin. We examined her genetic phenotype and detected anhaptoglobinemia, which occurs because of an allelic deletion in the Hp gene cluster. The presence of an antibody against haptoglobin was detected by performing ELISA. To prevent anaphylactic reactions, apheresis platelets were transfused after washing. Consequently, anaphylactic transfusion reactions did not develop. Here, we report the first case of anhaptoglobinemia causing anaphylactic transfusion reaction in Korea.
Alleles ; Anaphylaxis/*etiology ; Antineoplastic Agents/therapeutic use ; Female ; Gene Deletion ; Haptoglobins/*genetics/immunology ; Humans ; Isoantibodies/immunology ; Leukemia, Promyelocytic, Acute/complications/*diagnosis/drug therapy ; Middle Aged ; Phenotype ; Platelet Transfusion/*adverse effects ; Recurrence ; Republic of Korea ; Thrombocytopenia/complications/diagnosis

Adult ; Antibodies, Monoclonal, Murine-Derived ; administration & dosage ; therapeutic use ; Connective Tissue Diseases ; complications ; Female ; Humans ; Lung Diseases ; complications ; Middle Aged ; Rituximab ; Thrombocytopenia ; drug therapy ; etiology ; Young Adult

Immune thrombocytopenia (ITP) is a common acquired hemorrhagic disease. Conventional view considered its pathogenesis as the destruction of platelets induced by platelet associated antibodies, the target of treatment are inhibiting the production of antibodies and blocking the destruction of platelets in reticuloendothelial system, but they are ineffective in part of ITP patients, who transform to chronic/refractory ITP (C/RITP). As to children's C/RITP, the effect of first-line therapy is low, while the second-line therapy isn't effective definitely and has obvious side effects. The safe and effective second-line drugs to prevent disease progressing are urgently required. Recently, a pathogenesis that decrease the platelet production has been confirmed, thrombopoietic drugs, including thrombopoietin (TPO) and its receptor agonist (TRA), are under research and clinical application gradually. Recombinate human TPO (rhTPO) has accomplished Phase III clinical trails in adult C/RITP and tumor children. The Phase III clinical trails of romiplostim and eltrombopag, as the representative of TRA, in adult C/RITP have been performed. There are also two clinical trails of TRA for children's C/RITP, the efficacy and safety have been approved, with the convenience for using. In pediatric population, they have a good clinical application. In this article the research and development of thrombopoietic drugs and their perspective in pediatric clinical use are reviewed.
Child ; Clinical Trials, Phase III as Topic ; Humans ; Thrombocytopenia ; drug therapy ; etiology ; Thrombocytopenia, Neonatal Alloimmune ; drug therapy ; Thrombopoietin ; therapeutic use

OBJECTIVETo evaluate the efficacy and toxicity of Yanshu injection (a compound Chinese traditional medicine from Sophora flauescens Ait) combined with concomitant radiochemotherapy in patients with stage III nasopharyngeal carcinoma.

METHODSSixty patients with stage III nasopharyngeal carcinoma were randomized into Yanshu group and control group (n = 30, each). Patients in the Yanshu group received Yanshu injection in addition to intensity modulated radiation therapy (IMRT) and concomitant chemotherapy, and those in the control group were treated with IMRT and concurrent chemotherapy.

RESULTSThe 1-year, 2-year, 3-year and 4-year overall survival rates were 100%, 93.3%, 86.7%, 80.0% for Yanshu group, and 96.7%, 90.0%, 83.3%, 76.7% for the control group, respectively, with no significant difference between the two groups (P = 0.565). The 1-year, 2-year, 3-year and 4-year progression-free survival rates were 96.7%, 90.0%, 83.3%, 70.0% for Yanshu group, and 90.0%, 86.7%, 76.7%, 66.7% for control group, respectively, with no significant difference (P = 0.554). However, the reaction of mucosa of oral cavity, myelosuppression and thrombocytopenia in the Yanshu group were significantly lower than that in the control group (P < 0.05). The quality of life of the patients in the Yanshu group was significantly higher than that in the control group (P < 0.05).

CONCLUSIONSYanshu injection combined with radiochemotherapy in patients with stage III nasopharyngeal carcinoma show a good efficacy and can reduce the side effects of radiochemotherapy of nasopharygeal carcinoma, and improve the quality of life of the patients.

Antineoplastic Combined Chemotherapy Protocols ; adverse effects ; therapeutic use ; Carcinoma, Squamous Cell ; drug therapy ; pathology ; therapy ; Chemoradiotherapy ; methods ; Cisplatin ; administration & dosage ; Combined Modality Therapy ; Disease-Free Survival ; Drugs, Chinese Herbal ; adverse effects ; isolation & purification ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Leukopenia ; chemically induced ; etiology ; Male ; Medicine, Chinese Traditional ; Mucositis ; chemically induced ; etiology ; Nasopharyngeal Neoplasms ; drug therapy ; pathology ; therapy ; Neoplasm Staging ; Paclitaxel ; administration & dosage ; Plants, Medicinal ; chemistry ; Quality of Life ; Radiotherapy, Intensity-Modulated ; adverse effects ; Sophora ; chemistry ; Survival Rate ; Thrombocytopenia ; chemically induced ; etiology