Therapeutic Plasma Exchange in a Patient with Hemophagocytic Lymphohistiocytosis

A 22-year old female patient with systemic lupus erythematosus presenting microangiopathic hemolytic anemia was treated with therapeutic plasma exchange 23 times. The patient's condition and laboratory findings (aspartate aminotransferase, alanine aminotransferase, ferritin, total bilirubin, and lactate dehydrogenase) did not improve despite the initial 18 therapeutic plasma exchange treatments. Thrombotic thrombocytopenic purpura was ruled out due to normal ADAMTS-13 activity test result; hemophagocytic lymphohistiocytosis was diagnosed based on fever, splenomegaly, pancytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow aspiration. The patient's condition improved rapidly upon treatment with a combination of immunosuppressants and cytotoxic agents, and more therapeutic plasma exchanges were performed five consecutive times with prolonged intervals in between. We observed that therapeutic plasma exchange treatment alone was not effective enough to treat hemophagocytic lymphohistiocytosis, unlike thrombotic thrombocytopenic purpura. Therefore, it is necessary to determine and start drug administration promptly in the treatment of hemophagocytic lymphohistiocytosis with thrombotic microangiopathy.

Therapeutic Leukapheresis for the Management of Hyperleukocytosis: Ten-year Experience in a Tertiary Care Hospital

BACKGROUND: Hyperleukocytosis is a medical emergency that is characterized by increased blood viscosity and predisposition to various neurological, pulmonary, and gastrointestinal complications. In addition, patients are at risk of the tumor lysis syndrome because of the increased tumor burden. Therapeutic leukapheresis is an important treatment for these emergent states. In this study, we retrospectively analyzed therapeutic leukapheresis procedures that were performed in our institution during the last 10 yr. METHODS: We retrospectively analyzed therapeutic leukapheresis procedures conducted from July 2005 to March 2015 at a tertiary care hospital. We present our observations, especially the procedural characteristics and hematological parameters before and after the aforementioned procedures. RESULTS: Seventy-two patients underwent a total of 146 therapeutic leukapheresis procedures. The average presenting white blood cell (WBC) count was 268×10(3)/µL, and ranged from 54×10(3)/µL to 673×10(3)/µL. After an average of two sessions, a statistically significant drop in the WBC counts was observed. The average WBC removal rates during the initial and entire therapeutic leukapheresis procedures of each patient were 33% and 46%, respectively. The platelet count and hemoglobin concentration were significantly reduced. CONCLUSIONS: Therapeutic leukapheresis significantly reduces peripheral WBC counts and is a safe and effective procedure for the treatment of hyperleukocytosis.

A Choice and Precautions of Replacement Fluids for Therapeutic Plasma Exchange / 대한수혈학회지

BACKGROUND: Therapeutic plasma exchange (TPE) is an effective and practical treatment for separation and removal of harmful antibodies or pathogenic substances from the blood. The volume of plasma removed must be replaced by a replacement fluid such as 4~5% albumin solution or Fresh frozen plasma (FFP). We conducted a study of coagulopathy using albumin solution and checked the chemical composition of fresh frozen plasma. METHODS: We measured pre- and post-TPE PT/aPTT for evaluation of the effect of albumin replacement on coagulation from 192 TPE sessions of 19 patients. We also investigated routine chemistry test items including glucose and electrolytes from 10 randomly selected FFP. RESULTS: The post PT and aPTT within four hours after TPE were prolonged due to a transient decrease in coagulation factors, but were normalized within 2 days after TPE. All coagulation time was corrected to the level of the pre-TPE status within four hours before the next TPE except the patients who received TPE 6 times or more. FFP showed higher level in glucose, sodium and inorganic phosphate. CONCLUSION: Albumin exchange produces temporary coagulation factor deficiency. However, this transient factor deficiency rarely causes clinical problems and the factors are rapidly corrected by redistribution and resynthesis. We should be careful about hypocalcemia, hyperglycemia, and hypernatremia when using FFP replacement.

High Dose Chemotherapy and Autologous Peripheral Blood Stem Cell Transplantation in Pediatric Patients with High-risk Neuroblastoma / 대한수혈학회지

BACKGROUND: High dose chemotherapy followed by autologous peripheral blood stem cell transplantation (PBSCT) has become standard therapy for high-risk neuroblastoma patients. We performed a retrospective analysis to assess the characteristics of peripheral blood stem cell harvest (PBSCH) and PBSCT and its clinical outcome. METHODS: We reviewed 17 cases of patients with high-risk neuroblastoma that underwent PBSCH and/or high dose chemotherapy followed by PBSCT. RESULTS: Sixteen patients had stage IV neuroblastoma and one patient had a stage III neuroblastoma with MYCN amplification. The median age of the 17 patients was 43 months (range 22~114 months) and the median body weight was 15 kg (range 10~24 kg). After induction chemotherapy using a modified N7 protocol, 34 PBSCHs (1.5 leukapheresis per PBSCH) were performed. A statistically significant correlation was found between the pre-leukapheresis CD34+ cell count and the total number CD34+ cells of the harvested products (P<0.0001). Tyrosine hydroxylase mRNA was not detected by RT-PCR in all of the leukapheresis products. High dose chemotherapy followed by PBSCT was performed in 24 cases. The mean infused CD34+ cell dose was 4.01x106/kg and WBC and platelet engraftment was performed on day 12.0 and 21.5, respectively. Eleven patients died, and six patients are surviving 11 to 68 months after PBSCT (median survival time, 35 months). CONCLUSION: In this single institution study, treatment with high dose chemotherapy and PBSCT was performed successfully for children with high-risk neuroblastoma.