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Therapeutic outcomes using subcutaneous low dose alemtuzumab for acquired bone marrow failure conditions.
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Therapeutic outcomes using subcutaneous low dose alemtuzumab for acquired bone marrow failure conditions.

Br J Haematol. 2017 Sep 14;:

Authors: Thota S, Patel BJ, Sadaps M, Balasubramanian S, Sanikommu S, Hirsch C, Marotta S, Sekeres MA, Risitano AM, Maciejewski JP

PMID: 28905372 [PubMed - as supplied by publisher]




Interim FDG-PET has no value in selecting patients who require treatment modification in both early- and advanced-stage Hodgkin lymphoma.
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Interim FDG-PET has no value in selecting patients who require treatment modification in both early- and advanced-stage Hodgkin lymphoma.

Br J Haematol. 2017 Sep 14;:

Authors: Adams HJA, Kwee TC

PMID: 28905368 [PubMed - as supplied by publisher]




Iron storage in liver, bone marrow and splenic Gaucheroma reflects residual disease in type 1 Gaucher disease patients on treatment.
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Iron storage in liver, bone marrow and splenic Gaucheroma reflects residual disease in type 1 Gaucher disease patients on treatment.

Br J Haematol. 2017 Sep 14;:

Authors: Regenboog M, Bohte AE, Akkerman EM, Stoker J, Hollak CEM

Abstract
Gaucher disease (GD) is a lysosomal storage disorder characterized by the storage of glycosphingolipids in macrophages. Despite effective therapy, residual disease is present in varying degrees and may be associated with late complications, such as persistent bone or liver disease and increased cancer risk. Gaucher macrophages are capable of storing iron and locations of residual disease may thus be detectable with iron imaging. Forty type 1 GD (GD1) patients and 40 matched healthy controls were examined using a whole-body magnetic resonance imaging protocol consisting of standard sequences, allowing analysis of iron content per organ, expressed as R2* (Hz). Median R2* values were significantly elevated in GD1 patients as compared to healthy controls in liver [41 Hz (range 29-165) vs. 38 Hz (range 28-53), P < 0·01], femoral bone marrow [54 Hz (range 37-129) vs. 49 Hz (range 39-69), P = 0·036] and vertebral bone marrow (118 Hz (range 82-210) vs. 105 Hz (range 76-149), P < 0·01). In the spleen, primarily focal Gaucher lesions known as Gaucheroma were found to have increased R2* values. R2* values of liver, spleen and vertebral bone marrow strongly correlated with serum ferritin levels. GD1 patients with persistent hyperferritinaemia demonstrate increased iron levels in liver and bone marrow, which may carry a risk for liver fibrosis and cancer.

PMID: 28905365 [PubMed - as supplied by publisher]




A clinical risk score for pulmonary artery thrombosis during acute chest syndrome in adult patients with sickle cell disease.
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A clinical risk score for pulmonary artery thrombosis during acute chest syndrome in adult patients with sickle cell disease.

Br J Haematol. 2017 Sep 14;:

Authors: Winchenne A, Cecchini J, Deux JF, De Prost N, Razazi K, Carteaux G, Galacteros F, Habibi A, Bartolucci P, Melica G, Khellaf M, Michel M, Maitre B, Mekontso Dessap A

Abstract
Pulmonary artery thrombosis (PAT) is involved in lung vascular dysfunction during acute chest syndrome (ACS) complicating sickle cell disease (SCD). No clinical score is available to identify patients eligible for multi-detector computed tomography (MDCT) angiography during ACS. This retrospective study aimed to develop a risk score for PAT during ACS (PAT-ACS risk score). Patients with SCD were investigated by MDCT during ACS. A logistic regression was performed to determine independent risks factors for PAT and to build the PAT-ACS risk score. A total of 43 episodes (11·9%) of PAT were diagnosed in 361 episodes of ACS. Multivariate analysis identified four risk factors, which were included in the PAT-ACS risk score: a baseline haemoglobin >82 g/l, the lack of a triggering factor for ACS, a platelet count >440 × 10(9) /l and a PaCO2 <38 mmHg at ACS diagnosis. The area under the receiver operating characteristic curve for the PAT-ACS risk score was 0·74 (95% confidence interval [CI] 0·69-0·79) and differed from that of the revised Geneva score (0·63 (95% CI 0·58-0·69); P = 0·04). The negative predictive value of a PAT-ACS risk score ≥2 was 94%. In conclusion, we propose a simple clinical risk score to identify SCD patients at high risk of PAT during ACS.

PMID: 28905364 [PubMed - as supplied by publisher]