Post-hematopoietic stem cell transplant hemophagocytic lymphohistiocytosis or an impostor: Case report and review of literature.

HLH occurring after HSCT is a relatively rare disease. Many conditions may mimic or trigger HLH in post-HSCT period (eg, cytokine release syndrome, engraftment syndrome, graft rejection/failure, acute graft-vs-host disease, infections systemic inflammatory response syndrome/sepsis, and thrombotic microangiopathy). Moreover, this period is usually marked by febrile illness, cytopenia, and a "cytokine storm" leading to elevation of inflammatory biomarkers like ferritin and sCD25. These parameters overlap with the diagnostic criteria for HLH. Such confounding factors make the management of post-HSCT HLH quite challenging. We illustrate this critical issue with case report of a patient who was diagnosed with HLH after allogeneic HSCT for tAML. He received MP and CsA for HLH but VP-16 was not administered due to fear of severe myelosuppression. Fortunately, he responded well to treatment and remains in remission to date. We recommend caution while using HLH-94/HLH-2004 guidelines for the diagnosis and management of post-HSCT HLH. In this article, we pinpoint these issues with a brief review of all the pediatric cases and clinical studies of post-HSCT HLH along with a critical evaluation of its various diagnostic criteria. Finally, based on the limitations of current diagnostic criteria, we suggest a need for formulating disease-specific diagnostic criteria for post-HSCT HLH.

The role of continuous renal replacement therapy in the management of acute kidney injury associated with sinusoidal obstruction syndrome following hematopoietic cell transplantation.

Maintaining fluid balance, pre- and post-MA-HCT is essential and usually requires frequent administration of diuretics. Hepatic sinusoidal obstructive syndrome is potentially life-threatening, especially when associated with AKI and MOF. This study describes six patients who developed AKI-associated SOS and diuretic-resistant FO who subsequently underwent CRRT using standardized management guidelines for fluid balance post-HCT. Retrospective chart review was done for HCT patients between September 2011 and October 2013 at a tertiary care children's hospital. Thirty-four patients underwent MA-HCT in the study period. Six patients had SOS complicated by diuretic-resistant FO and underwent CRRT. Defibrotide was used in three patients. Median time on CRRT was 10.5 days. Sixty-six percent (N = 4 of 6) of patients had full resolution of SOS symptoms with a mortality rate of 34% (N = 2 of 6). Among patients who had full recovery of SOS symptoms, one patient developed AKI, end-stage renal diseases and underwent kidney transplantation 34-months post-HCT. Thus, of six included patients, two died and one developed ESRD with only 50% (N = 3 of 6) good outcome. Use of a standardized, evidence-based fluid balance protocol and early initiation of CRRT for HCT-related AKI/SOS was associated with good outcomes.

Complete Resolution of Lymphoid Interstitial Pneumonia in a Patient With Juvenile Myelomonocytic Leukemia Treated With Allogeneic Bone Marrow Transplant: Killing 2 Birds With 1 Stone.

Lymphoid interstitial pneumonia (LIP) is a rare disease characterized by benign reactive polyclonal proliferation of bronchus-associated lymphoid tissue after exposure to inhaled or circulating antigen(s), leading to a disease symptomatology similar to idiopathic interstitial pneumonia. Its association with diseases that are caused due to immune dysregulation (autoimmune diseases, congenital/acquired immunodeficiency, and allogeneic bone marrow transplant) and response to immunomodulatory/suppressive medications suggests an immunologic pathophysiology. Although LIP has been reported in association with lymphoproliferative diseases like Castleman disease, it has never been described in patients with leukemia. We report the first case of LIP in a patient with juvenile myelomonocytic leukemia (JMML) who was found to have a novel germline mutation of unknown significance in additional sex combs-like-1 (ASXL1) gene and a pathogenic somatic mutation of protein tyrosine phosphatase, nonreceptor type 11 (PTPN11) gene at diagnosis. The patient underwent a matched unrelated bone marrow transplant for JMML with complete resolution of JMML and LIP with no recurrence to date. We also emphasize the importance of considering LIP in differential diagnosis of pulmonary lesions seen in conjunction with hematologic malignancies and distinguishing it from malignant infiltration of the lung.

Azacitidine as a bridge to allogeneic hematopoietic cell transplantation in a pediatric patient with Fanconi anemia and acute myeloid leukemia.

HCT is the definitive therapy for patients with FA and AML. Conventional cytotoxic agents cause potential DNA damage, and currently, there is no established regimen for these patients prior to HCT. A 13-year-old male with FA and refractory AML was given azacitidine, achieved morphologic remission and underwent HCT. At 95 days after HCT, he relapsed. Azacitidine along with DLI was used as first salvage therapy. Azacitidine was overall well tolerated with minimal side effects. In patients with AML and FA, azacitidine can be considered an alternative to conventional chemotherapy.

Successful hematopoietic cell transplantation in a patient with X-linked agammaglobulinemia and acute myeloid leukemia.

X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by marked reduction in all classes of serum immunoglobulins and the near absence of mature CD19(+) B-cells. Although malignancy has been observed in patients with XLA, we present the first reported case of acute myeloid leukemia (AML) in a patient with XLA. We also demonstrate the complete correction of the XLA phenotype following allogeneic hematopoietic cell transplantation for treatment of the patient's leukemia.

Perspectives of people who are deaf and hard of hearing on mental health, recovery, and peer support.

This qualitative study sought to better understand the experiences of deaf and hard of hearing individuals with accessing recovery-oriented mental health services and peer support via a focus group and interviews. Cultural brokers were used to facilitate culturally-sensitive communication with study participants. Findings indicate that access to adequate mental health services, not just recovery-oriented and peer support services, is not widely available for this population, largely due to communication barriers. Feelings of isolation and stigma are high among this population. Public mental health systems need to adapt and expand services for various cultural groups to insure recovery.

Care Transformation Collaborative of Rhode Island: Building a Strong Foundation for Comprehensive, High-Quality Affordable Care.

As the Patient Centered Medical Home (PCMH) model has evolved nationally and in Rhode Island, there has been increased recognition that PCMH has not been sufficient to achieve desired cost and quality goals. In this article, we describe the evolving concept of "comprehensive primary care" in Rhode Island, which includes addressing the behavioral health and social determinants of health (SDOH) needs of patients. These needs are identified through systematic screening and dedicated care management and care coordination for patients who present with complex needs.

Patient-Centered Medical Home Adoption: Lessons Learned and Implications for Health Care System Reform.

Case studies of 8 primary care medical homes participating in a Massachusetts-based initiative were conducted to understand the approaches they used to operationalize medical home standards and associated barriers. All sites received their National Committee on Quality Assurance recognition as medical homes, yet varied considerably in how components were implemented. Despite this variation, they faced similar challenges to implementing and sustaining medical home standards. Variations and challenges strongly emerged in 4 areas: team-based care, scheduling and online access, identifying and managing high-risk patients, and organizing follow-up care. Our study offers insight into various pathways to medical home success, and notes areas for further study.

Clarifying the role of the mental health peer specialist in Massachusetts, USA: insights from peer specialists, supervisors and clients.

Mental health peer specialists develop peer-to-peer relationships of trust with clients to improve their health and well-being, functioning in ways similar to community health workers. Although the number of peer specialists in use has been increasing, their role in care teams is less defined than that of the community health worker. This qualitative study explored how the peer specialist role is defined across different stakeholder groups, the expectations for this role and how the peer specialist is utilised and integrated across different types of mental health services. Data were collected through interviews and focus groups conducted in Massachusetts with peer specialists (N = 44), their supervisors (N = 14) and clients (N = 10) between September 2009 and January 2011. A consensus coding approach was used and all data outputs were reviewed by the entire team to identify themes. Peer specialists reported that their most important role is to develop relationships with clients and that having lived mental health experience is a key element in creating that bond. They also indicated that educating staff about the recovery model and peer role is another important function. However, they often felt a lack of clarity about their role within their organisation and care team. Supervisors valued the unique experience that peer specialists bring to an organisation. However, without a defined set of expectations for this role, they struggled with training, guiding and evaluating their peer specialist staff. Clients reported that the shared lived experience is important for the relationship and that working with a peer specialist has improved their mental health. With increasing support for person-centred integrated healthcare delivery models, the demand for mental health peer specialist services will probably increase. Therefore, clearer role definition, as well as workforce development focused on team orientation, is necessary for peer specialists to be fully integrated and supported in care teams.

Overview of publicly funded managed behavioral health care.

Using MEDLINE and other Internet sources, the authors perform a systematic review of published literature. A total of 109 articles and reports are identified and reviewed that address the development, implementation, outcomes, and trends related to Managed behavioral health care (MBHC). MBHC remains a work in progress. States have implemented their MBHC programs in a number of ways, making interstate comparisons challenging. While managed behavioral health care can lower costs and increase access, ongoing concerns about MBHC include potential incentives to under-treat those with more severe conditions due to the nature of risk-based contracting, the tendency to focus on acute care, difficulties assuring quality and outcomes consistently across regions, and a potential cost-shift to other public agencies or systems. Success factors for MBHC programs appear to include stakeholder involvement in program and policy development, effective contract development and management, and rate adequacy.