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JAK2, CALR, MPL and ASXL1 mutational status correlates with distinct histologic features in Philadelphia chromosome-negative myeloproliferative neoplasms.
Related Articles

JAK2, CALR, MPL and ASXL1 mutational status correlates with distinct histologic features in Philadelphia chromosome-negative myeloproliferative neoplasms.

Haematologica. 2017 Nov 16;:

Authors: Wong WJ, Hasserjian RP, Pinkus GS, Breyfogle LJ, Mullaly A, Pozdnyakova O

PMID: 29146710 [PubMed - as supplied by publisher]




Alternatively spliced Fibronectin Extra Domain A is required for hemangiogenic recovery upon bone marrow chemotherapy.
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Alternatively spliced Fibronectin Extra Domain A is required for hemangiogenic recovery upon bone marrow chemotherapy.

Haematologica. 2017 Nov 16;:

Authors: Malara A, Gruppi C, Celesti G, Abbonante V, Viarengo G, Laghi L, De Marco L, Muro AF, Balduini A

PMID: 29146709 [PubMed - as supplied by publisher]




Inflammatory molecule reduction with hydroxyurea therapy in children with sickle cell anemia.
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Inflammatory molecule reduction with hydroxyurea therapy in children with sickle cell anemia.

Haematologica. 2017 Nov 16;:

Authors: Penkert RR, Hurwitz JL, Thomas P, Rosch J, Dowdy J, Sun Y, Tang L, Hankins JS

PMID: 29146708 [PubMed - as supplied by publisher]




Outcomes after allogeneic hematopoietic stem cell transplantation in patients with acute myeloid leukemia harboring t(7;11)(p15;p15).
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Outcomes after allogeneic hematopoietic stem cell transplantation in patients with acute myeloid leukemia harboring t(7;11)(p15;p15).

Haematologica. 2017 Nov 16;:

Authors: Harada K, Doki N, Aoki J, Mori J, Machida S, Masuko M, Uchida N, Najima Y, Fukuda T, Kanamori H, Ogawa H, Ota S, Ogawa K, Takahashi S, Kasai M, Maeda A, Nagafuji K, Kawakita T, Ichinohe T, Atsuta Y

PMID: 29146707 [PubMed - as supplied by publisher]




The clinical and laboratory evaluation of familial hemophagocytic lymphohistiocytosis and the importance of hepatic and spinal cord involvement: a single center experience.
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The clinical and laboratory evaluation of familial hemophagocytic lymphohistiocytosis and the importance of hepatic and spinal cord involvement: a single center experience.

Haematologica. 2017 Nov 16;:

Authors: Beken B, Aytac S, Balta G, Kuskonmaz B, Uckan D, Unal S, Cetin M, Gumruk F

Abstract
Familial hemophagocytic lymphohistiocytosis is an autosomal recessive, life-threatening condition characterized by defective immune response. A retrospective analysis was performed on 57 patients diagnosed with familial hemophagocytic lymphohistiocytosis at Hacettepe University Pediatric Hematology Department. Mutation analysis was performed on 37 patients and of these; 11 had UNC13D, 10 had PRF1 and 3 had STX11 gene mutation. Of these patients 44% were found to have central nervous system involvement on admission and spinal cord involvement was also seen in 5 patients. Remission was achieved in 24 patients with the treatment, in a median time of 76 days (min-max: 15-705 days). Time to get into remission was prolonged 3.1 times in patients with a ferritin level ≥1500 mg/dL. When patients were grouped according to age [Group 1 (≤ 2 years), Group 2 (>2 years)]; patients in Group 1 had higher ferritin and aspartate aminotransferase levels but lower fibrinogen levels. The five year survival rate was also lower in Group 1.When patients in Group 1were divided into two sub-groups according to hepatic involvement, the five year survival rate of patients who had hepatic involvement was significantly lower than those patients without hepatic involvement (0.7%, 27%, respectively) (p=0.002). The five year survival rate of patients with hematopoietic stem cell transplantation was significantly higher than the patients without it (44%, 16%, respectively) (p= 0.02). In conclusion, age being two years and under, ferritin level above 1500 mg/dL, spinal cord or hepatic involvement should be kept in mind as poor prognostic factors in familial hemophagocytic lymphohistiocytosis.

PMID: 29146706 [PubMed - as supplied by publisher]




Complement C3 is a novel modulator of the anti-FVIII immune response.
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Complement C3 is a novel modulator of the anti-FVIII immune response.

Haematologica. 2017 Nov 16;:

Authors: Rayes J, Ing M, Delignat S, Peyron I, Gilardin L, Vogel CW, Fritzinger DC, Frémeaux-Bacchi V, Kaveri SV, Roumenina LT, Lacroix-Desmazes S

Abstract
Development of neutralizing antibodies against therapeutic factor VIII (FVIII) is the most serious complication of the treatment of hemophilia A. Increasing evidence shows the multifactorial origin of the anti-FVIII immune response, combining both genetic and environmental factors. While a role for the complement system on innate as well as adaptive immunity has been documented, the implication of complement activation on the onset of the anti-FVIII immune response is unknown. Here, using in vitro assays for FVIII endocytosis by human monocyte-derived dendritic cells and presentation to T cells, as well as animal models of in vivo complement depletion, we show a novel role for complement C3 in enhancing the immune response against therapeutic FVIII. In vitro, complement C3 and its cleavage product C3b enhanced FVIII endocytosis by dendritic cells and presentation to a FVIII-specific CD4+ T-cell hybridoma. The C1 domain of FVIII was previously shown to play an important role in FVIII endocytosis, and alanine substitutions of the K2092, F2093 and R2090 C1 residues drastically reduces FVIII uptake in vitro. Interestingly, complement activation rescued the endocytosis of the FVIII C1 domain triple mutant. In a mouse model of severe hemophilia A, transient complement C3 depletion by humanized cobra venom factor, which does not generate anaphylatoxin C5a, significantly decreased the primary anti-FVIII immune response, while it did not affect the immune response to A Disintegrin And Metalloprotease with ThromboSpondin type I-repeats-13. Taken together, our results suggest an important adjuvant role for the complement cascade in the immune response to therapeutic FVIII.

PMID: 29146705 [PubMed - as supplied by publisher]