Extracorporeal photopheresis as part of a multimodality approach yields a high durable clinical response in patients with erythrodremic MF and SS. The kinetics of the response differ between the blood and skin. The blood response occurs earlier and lasts longer; it does not necessarily predict the clinical skin response. Further studies are needed to determine if there is a survival advantage to a blood response in the absence of a skin response.
Background: Hereditary spherocytosis (HS) is the most common inherited hemolytic anemia. The flow cytometric test using eosin-5 0 maleimide (EMA) is a well-established diagnostic method. However, in order to improve HS detection, it is recommended that EMA and an osmotic fragility test (OFT) both be performed. OFT is time consuming and labor intensive. We used a flow cytometric (FOFT) adaptation of the classical OFT reported by Yamamoto. We compare the FOFT to the classical OFT including practical data and propose options for simplifying this method.Methods: Suspected and known HS patients and controls were tested by the following methods: EMA, OFT, and FOFT including some modifications.
Results:The FOFT method is robust and correlates to loss of red blood cells. OFT and FOFT gave similar results in healthy controls and four HS patients. Normal range for FOFT in 70 adults is shown and can be used as a reference value. Neonates should have their own normal range defined. Overnight sample incubation at 37 C did not add information to the FOFT results. Conclusion: Our modified Yamomoto FOFT can replace the classic OFT as the addition to EMA for the diagnosis of HS. The use of flow cytometry in both these methods requires small sample volume, is reproducible, simpler, and produces results more rapidly.
Histiocytic sarcoma (HS) is a rare, malignant, and aggressive subtype of histiocytosis. We present an unusual case of aggressive HS presenting in the gastrointestinal tract and gallbladder that progressed after several lines of chemotherapy with a leukemic phase. We review the clinical, pathological, and molecular characteristics of HS in this case and review the literature on HS involving the digestive system as well as on overt leukemic phase of this disease. HS is often diagnosed at an advanced stage, and mortality is high. We discuss the therapeutic approach to patients with HS. We highlight the role of overexpression and somatic alterations in the RAF-MEK-ERK pathway in the pathogenesis of HS and discuss potential targeted approaches to treat these rare tumors.
Background: Fibrinogen-like protein 2 (FGL2) is a serine protease capable of converting prothrombin into thrombin (i.e., prothrombinase activity) while bypassing the classic coagulation cascade. It has been reported to be expressed by mononuclear blood cells and endothelial cells. There are multiple reports that FGL2 supports tumor development and metastasis. However, in the blood, the origin or functional significance has not been established.Objective: To identify the origin of the FGL2 prothrombinase, a malignancy related enzyme, in peripheral blood cells.Methods: Peripheral blood samples were collected in complete blood count tubes. Blood cells and platelets were thoroughly washed to produce plasma-free samples. Procoagulant activity was measured in the samples' extracts using a thrombin generation test or an adjusted prothrombin time (PT) test in plasma deficient factor X. Results were further supported by confocal microscopy, immunoprecipitation, flow cytometry, enzyme-linked immunosorbent assays and specific inhibition assays. Results: The pro-coagulant activity of FGL2 was detected in purified platelets but not in white blood cells. FGL2 protein was readily detected in platelets. Quiescent platelets were shown to store FGL2 protein in an active form. Upon activation, platelets secreted the active FGL2 into the milieu. Conclusions: Active FGL2 prothrombinase is stored in platelets. This suggests the involvement of platelets in malignancies.
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