Daniel K. Partain

Vancomycin-resistant Enterococcus colonization and bloodstream infection: prevalence, risk factors, and the impact on early outcomes after allogeneic hematopoietic cell transplantation in patients with acute myeloid leukemia.

Screening for vancomycin‐resistant Enterococcus (VRE) is performed at many transplant centers, but data on the impact of VRE colonization and bloodstream infection (BSI) on hematopoietic cell transplantation (HCT) outcomes remain conflicting.

Allogeneic hematopoietic stem cell transplant overcomes the adverse survival effect of very high risk and unfavorable karyotype in myelofibrosis

The prognostic importance of genetic information in primary myelofibrosis (PMF) was recently highlighted in a study of over 1000 cytogenetically‐annotated patients; 5‐year survival rates were 8% for very high risk (VHR), 27% “unfavorable” and 45% “favorable” karyotype. The current study addresses the practice‐relevant question of whether or not allogeneic hematopoietic stem cell transplant (HCT) can overcome the detrimental survival effect of VHR or unfavorable karyotype. The study included 67 patients with PMF or secondary MF who received HCT at the Mayo Clinic and in whom pretransplant cytogenetic information was available. Dynamic international prognostic scoring system (DIPSS) risk distribution was 13% high, 66% intermediate‐2 and 21% intermediate‐1. Cytogenetic risk distribution was 11% VHR, 34% unfavorable and 55% favorable. At median post‐HCT follow‐up of 60 months for living patients (range 34‐170), 28 (42%) deaths were recorded. Five‐year survival was 62% and was not affected by VHR or unfavorable karyotype (P = .68). The salutary effect of HCT in patients with VHR or unfavorable karyotype was also apparent during analysis of a combined dataset that included a nontransplant cohort of 383 patients with PMF; multivariable analysis of the combined dataset (n = 450) resulted in HRs (95% CI) of 2.4 (1.6‐3.6) for absence of transplant, 3.3 (2.2‐4.8) for VHR karyotype, 1.6 (1.2‐2.1) for unfavorable karyotype, 2.9 (2.0‐4.2) for DIPSS high and 1.7 (1.4‐2.2) for DIPSS intermediate‐2. These observations were further confirmed by analysis of more stringently matched case‐control subset cohorts and provide the evidence for the therapeutic preference of HCT in cytogenetically high risk patients with MF.

Comparison of reduced intensity conditioning regimens used in patients undergoing hematopoietic stem cell transplantation for myelofibrosis

The aim of this study is to compare clinical outcomes of patients who underwent allogeneic stem cell transplantation (HCT) for myelofibrosis with reduced intensity conditioning (RIC) using either Busulfan Fludarabine (BuFlu), Fludarabine Bis-chlorethyl-nitroso-urea/ carmustine Melphalan (FBM) or Fludarabine Melphalan (FluMel) regimens. Sixty-one patients were identified who underwent HCT with one of these RIC regimens. Overall survival (OS) was not different in the 3 groups. However, 100% donor chimerism was seen in more frequently at day +30 and day +100 in patients who received FBM or FluMel than BuFlu, in both CD3 and CD33 fractions. For instance, 100% donor chimerism in CD33 fraction was present in 100% patients in FBM cohort, 90% in FluMel cohort while 44% in BuFlu cohort at day +100. Acute graft-versus host disease, grade 2–4 and grade 3–4, was not statistically different in the 3 groups (BuFlu 47 and 35%, FBM 68 and 27%, FluMel 68 and 46%; p = 0.31 and 0.45). Relapses and non-relapse mortality was also not statistically significantly different. Our study shows similar OS with these 3 RIC regimens in myelofibrosis; although donor chimerism at day +30 and day +100 was better in patients who received FBM and FluMel.

Providing Appropriate End-of-Life Care to Religious and Ethnic Minorities

There is overwhelming evidence that racial and ethnic minorities face multiple health care disparities. Recognizing and addressing cultural and religious/spiritual (RS) values is a critical aspect of providing goal-concordant care for patients facing a serious illness, especially at the end of life. Failure to address a patients cultural and RS needs can lead to diminished quality of care and worse health outcomes. Given the multitude of cultural and RS values, we believe that a framework of cultural and RS curiosity along with a willingness to engage patients in discussions about these elements of their care within an interdisciplinary team should be the goal of all providers who are discussing goals, preferences, and values with patients facing advanced terminal illness.

72-Year-Old Woman With Redness, Swelling, and Pain of the Forearms and Hands

dent tious A 72-year-old woman from far eastern Kansas who had a history of psoriatic arthritis treated with methotrexate and infliximab presented to the Mayo Clinic Hospital, Saint Marys Campus emergency department for evaluation of redness, swelling, and pain of both forearms and hands of 10 weeks’ duration. Psoriatic arthritis had been diagnosed 4 years earlier, and her disease had been under good control until 3 months previously. Six weeks before the current presentation, the patient’s rheumatologist stopped her methotrexate, started prednisone at 20 mg/d (tapered to 2.5 mg/d), and increased the frequency of her infliximab infusions from every 6 weeks to every 4 weeks in the setting of worsening hand pain, swelling, and redness. Two weeks before presenting to the emergency department, redness, swelling, and induration of her forearms developed. Her right hand grew increasingly painful, and black skin discoloration developed over the right thumb pad. On presentation, her vital signs were notable for a heart rate of 116 beats/min and respirations of 25/min. Physical examination revealed diffuse edema of both forearms and hands with overlying erythematous, indurated plaques on the left forearm without palpable tenderness or crepitus. Marked swelling, induration, erythema, warmth, and scaling were noted on the thenar eminence and palmar metacarpophalangeal joint line of the right hand. There were 2 discrete areas of ulcerated black, dry, nonmalodorous skin on the right thumb pad. Laboratory studies yielded the following findings (reference ranges provided parenthetically): leukocytes, 10.5 10/L (3.5-10.5 10/L) with 54% neutrophils; plasma lactate, 3.3 mmol/L (0.6-2.3 mmol/L); and C-reactive protein, 128.2 mg/L ( 8.0 mg/L). Plain radiography of the hands revealed severe erosive osteoarthritis, chondrocalcinosis, and no subcutaneous gas.

Spur cell anemia in the setting of progressive liver allograft failure

We report a case of a 44-year-old female with a history of orthotopic liver transplant for presumed primary sclerosing cholangitis/autoimmune hepatitis overlap in 1994. In 2015, she developed acute worsening of chronic anemia with evidence of hemolysis (indirect hyperbilirubinemia, reticulocytosis, low haptoglobin), and was initially felt to have microangiopathic hemolytic anemia secondary to tacrolimus. She required 3–4 units of packed red blood cells per week, and she received plasma exchange, rituximab, and eculizumab without improvement. A few months later, she was admitted to the hospital with progressive decline in mental status determined to be secondary to hepatic encephalopathy. Hemoglobin was 9.0 g/dL on admission and 7.1 g/dL on hospital day 2. Peripheral blood smear demonstrated marked anisopoikilocytosis with acanthocytes (spur cells). The patient underwent vibration controlled transient elastography of the liver consistent with cirrhosis. Her model for end-stage liver disease (MELD) score at hospital discharge was 30, and she is currently undergoing expedited workup for repeat liver transplant. Spur cell anemia is a rare cause of anemia that has traditionally been described as a consequence of alcoholic liver cirrhosis [1] but has recently been described in patients with advanced liver disease from other causes [2]. Spur cells form as a consequence of abnormal cholesterol to phospholipid ratios in red cell membranes caused by decreased apolipoproteins in the setting of liver cirrhosis [3–5]. Spur cells are susceptible to splenic sequestration and destruction, and patients are generally refractory to blood transfusions because transfused cells rapidly acquire the abnormal membrane configuration [6]. The only effective treatment is liver transplantation [7].