Mycosis fungoides in pediatric patients: Clinical features, diagnostic challenges, and advances in therapeutic management.

Mycosis fungoides (MF) is the most common primary cutaneous lymphoma in pediatric patients. Given the indolent nature of MF, symptoms often present in childhood but may not be diagnosed as MF until adulthood. Delayed diagnosis is associated with poor long-term prognosis. Thus, increased clinician recognition and accurate diagnosis of early-stage MF in pediatric patients is critically important. In this review, we summarize the clinical features of the most common pediatric MF subtypes and highlight important differences between pediatric and adult MF. Moreover, we reviewed all pediatric MF case series published between 2008 and 2018 to analyze treatment modalities and identify emerging therapies. As treatment of pediatric MF is complex, selection of therapy varies significantly depending upon the specific clinical characteristics, disease severity, and patients' preferences.

Racial Disparities in the Clinical Presentation and Prognosis of Patients with Mycosis Fungoides.

OBJECTIVE: Racial and gender disparities in mycosis fungoides (MF) are understudied. The objective of this study was to test the hypothesis that worse prognosis in blacks with MF is mediated by higher disease stage at diagnosis and by earlier disease onset in black females. METHODS: We conducted retrospective chart review of 337 patients with clinically-suspected MF seen at Johns Hopkins between 2003 and 2018, requiring biopsy-proven disease for study inclusion. Patient demographics, initial stage/percent body surface area (BSA) involvement, pathology type, flow cytometry results, and treatment regimens were recorded. RESULTS: Of 303 patients with confirmed MF, 166 (55%) were white, 107 (35%) black, 10 (3.3%) Middle Eastern, 6 (2.0%) Asian, and 14 (4.6%) Hispanic/other. Blacks were 3 times as likely (95% CI: 1.2, 8.0) to have Stage 2 disease to have Stage 2 disease at diagnosis as compared to whites as whites. In females, blacks were younger at diagnosis (p = 0.003) and at death (p = 0.008) compared to whites. In males, blacks had 4 times the odds of late-stage disease (p = 0.017) and presented with 19% greater BSA involvement on average compared to whites (p < 0.001). CONCLUSIONS: Compared to their white counterparts in this cohort, black males were diagnosed with MF at a higher stage with greater skin involvement while black females were diagnosed and died earlier. Earlier recognition of MF in skin of color and closer follow-up of black females with early-onset, aggressive disease may help to mitigate disparities in outcomes.

Comorbidities in Mycosis Fungoides and Racial Differences in Co-Existent Lymphomatoid Papulosis: A Cross-Sectional Study of 580 Patients in an Urban Tertiary Care Center.

Background: Mycosis fungoides (MF) is a cutaneous T-cell lymphoma. Previous reports have suggested MF is associated with inflammatory conditions such as psoriasis, increased cardiovascular risk factors as well as secondary neoplasms. Methods: A cross-sectional study of MF patients seen from 2013 to 2019 was performed. Comorbidities were selected based on the 2015 Medicare report highlighting the most common chronic medical illnesses in the United States. Lifetime comorbidity occurrence in patients with MF were compared with that in patients with atopic dermatitis, psoriasis and patients without MF. Additional analyses were performed with patients sub-stratified by race. Results: Compared to control groups, MF was strongly associated with lymphomatoid papulosis and Hodgkin's disease, but not significantly associated with lung, breast or colon cancer. Interestingly, the association with lymphomatoid papulosis was observed in Caucasians (CI 1062-4338; p < 0.001) and not African Americans (p = 0.9). Patients with MF had a greater association with congestive heart failure, hypertension (HT) and hyperlipidemia (HLD) compared with the general population. However, they were significantly less likely to have HT and HLD when compared with psoriasis patients (HT CI: 0.6-0.9; p < 0.001, and HLD CI: 0.05-0.07; p < 0.001). MF patients were also significantly less likely to have concomitant vitamin D deficiency compared with atopic dermatitis (AD) and psoriasis (p < 0.001). Conclusions: Our results suggest that the association of MF with lymphomatoid papulosis varies by race. Compared to the general population, hypertension and hyperlipidemia were positively associated with MF, however, these were significantly less likely on comparison to psoriasis. Unlike previously described, vitamin D deficiency was found to be significantly less in patients with MF.

Aprepitant for the Treatment of Chronic Refractory Pruritus.

Chronic pruritus is a difficult condition to treat and is associated with several comorbidities, including insomnia, depression, and decreased quality of life. Treatment for chronic itch includes corticosteroids, antihistamines, and systemic therapies such as naltrexone, gabapentin, UV light therapy, and immunomodulatory treatments, including azathioprine, methotrexate, and cellcept. However, some patients still remain refractory to conventional therapy. Aprepitant is a neurokinin-1 receptor antagonist approved for the prevention of chemotherapy-induced and postoperative nausea and vomiting (CINV, PONV). Recently, aprepitant has demonstrated effectiveness in several case series and open label trials in relieving pruritus for patients refractory to other treatments. Patients with pruritus associated with Sézary syndrome, mycosis fungoides, lung adenocarcinoma, breast carcinoma, sarcomas, metastatic solid tumors, chronic kidney disease, hyperuricemia, iron deficiency, brachioradial pruritus, and Hodgkin's lymphoma have experienced considerable symptom relief with short-term use of aprepitant (up to two weeks). Due to differences in reporting and evaluation of drug effects, the mechanism of aprepitant's role is difficult to understand based on the current literature. Herein, we evaluate aprepitant's antipruritic effects and discuss its mechanism of action and adverse effects. We propose that aprepitant is an alternative for patients suffering from pruritus who do not obtain enough symptom relief from conventional therapy.

Regression of cutaneous invasive squamous cell carcinoma in a patient with chronic cutaneous graft versus host disease.

Numerous complications can be observed in the post-transplant period among recipients of hematopoietic stem cells including graft-versus-host disease (GVHD), which is associated with significant morbidity and mortality. On the other hand, graft versus tumor (GVT) effect is a well-described phenomenon in patients with hematologic malignancies and has also been reported in renal cell cancer, ovarian cancer, breast carcinoma, and melanoma. We describe spontaneous regression of a cutaneous invasive squamous cell carcinoma and multifocal atypical intraepidermal proliferations in a patient with chronic graft-versus-host disease following initiation of extracorporeal photopheresis (ECP). This observation raises questions regarding the GVT in cutaneous neoplasms and potential immunomodulatory effects of ECP.

The use of blind skin biopsy in the diagnosis of intravascular B-cell lymphoma.

Intravascular B-cell lymphoma is a rare type of non-Hodgkin's lymphoma that is characterized by a clonal proliferation of lymphoblasts within small blood vessels. Patients present with nonspecific symptoms and are often only given a diagnosis at autopsy. We report a case of intravascular B-cell lymphoma, characterized by pyrexia, anemia, thrombocytopenia, and mental status decline, without obvious cutaneous manifestations, that was diagnosed with blind skin biopsy.