Citations

Multiple myeloma is a malignant neoplasm of plasma cells that accumulate in bone marrow, leading to bone destruction and marrow failure. This manuscript discusses the management of patients with solitary plasmacytoma, smoldering multiple myeloma, and newly diagnosed multiple myeloma.

AbstractWe studied 2,528 patients with upfront autologous haematopoietic cell transplantation (AHCT) for multiple myeloma (MM) from 2008–2017 to develop a prognostic model to predict outcomes. High‐risk cytogenetics included t(4;14), t(14;16), t(14;20), del13q on karyotype, del17p, +1q or 1pdel. A Cox model identified factors prognostic of progression/relapse in a training subset (n = 1,246). A weighted score using these factors was assigned to a validation cohort (n = 774). Presence of high‐risk cytogenetics [hazard ratio, (HR) 1·68 (1·3–2·17)] and pre‐AHCT bone marrow plasma cells (BMPCs) ≥10% [1·68 (1·33–2·12)] were assigned 4 points each; albumin at diagnosis <3·5 g/dl [1·31 (1·07–1·61)] 2; standard risk cytogenetics 1, and no cytogenetics abnormality, BMPCs <10% at AHCT and albumin ≥3·5 g/dl at diagnosis 0 points each. A three‐category system with low risk (0–3), intermediate risk (4–8) and high risk (9–10) showed 3‐year progression‐free survival in the low vs. intermediate vs. high risk of 58% (95% CI: 52–63) vs. 49% (95% CI: 43–56) vs. 31% (95% CI: 12–51), P < 0.001 respectively, and 3‐year OS in low vs. intermediate vs. high risk of 88% (95% CI: 84–91) vs. 81% (95% CI: 76–86) vs. 64% (95% CI: 39–80); P < 0·001. Our prognostic scoring system can identify MM patients at risk for early relapse after AHCT.

PolyQ ATXN3 is a pharmacodynamic marker for SCA3, and a SNP associated with the expanded allele may serve to specifically target mutant ATXN3 .

AbstractBackground/ObjectivesThough maculopapular cutaneous mastocytosis is the most common form of pediatric mastocytosis, it remains unclear which patients will experience severe symptoms. We sought to better define the presentation and the cutaneous and systemic signs and symptoms in patients with maculopapular cutaneous mastocytosis.MethodsWe analyzed retrospective data on 227 patients diagnosed with maculopapular cutaneous mastocytosis prior to age 15 years from five US clinical sites. We collected data on signs, symptoms, age of onset, and laboratory testing.ResultsMedian age of onset of maculopapular cutaneous mastocytosis was 3 months, with 94% of patients presenting prior to age 2 (range 0‐15 years). Patients presenting before age 2 had significantly lower serum tryptase level (P = .019). Greater number of skin lesions (P = .006), number of reported skin signs and symptoms (P < .001), and higher tryptase levels (P < .001) were associated with more systemic symptoms.ConclusionChildren with maculopapular cutaneous mastocytosis, who have greater skin involvement, higher serum tryptase level, and more skin signs and symptoms, are more likely to have systemic symptoms.

Background and objectives The costoclavicular brachial plexus block is performed deep and posterior to the midpoint of the clavicle. There are limited data evaluating the spread of the costoclavicular brachial plexus block. We performed a cadaveric study to evaluate the spread of injectate after a costoclavicular brachial plexus block. Methods Five ultrasound-guided costoclavicular block injections were performed with 20 mL of 0.1% methylene blue. The brachial plexus and its branches were dissected from the level of C4 to the lower axilla. The extent of dye spread was recorded including spread to the phrenic nerve, suprascapular nerve, roots, trunks, divisions, cords and terminal branches of the brachial plexus. Results The dye extended cephalad to the level of the cricoid cartilage in two of the five injections; three injections had dye extending 0.75 cm, 1.5 cm and 2 cm caudad to the level of the cricoid cartilage, respectively. The C7, C8 and T1 nerve roots were stained in all injections. The dye did not extend cephalad to the C5 and C6 nerve roots. All trunks, cords and divisions of the brachial plexus were stained, as was the suprascapular nerve. There was no spread of dye to the phrenic nerve in any of the specimens. Conclusions This cadaveric study demonstrates that ultrasound-guided injection in the costoclavicular space spreads cephalad to the brachial plexus in the supraclavicular space, consistently reaching the suprascapular nerve and all trunks and cords of the brachial plexus, while sparing the phrenic nerve.