RESUMEN
Sézary syndrome (SS) is a form of cutaneous T-cell lymphoma (CTCL), demonstrating leukemic involvement of malignant T-cells. Known systemic sequelae of SS include hemophagocytic syndrome-induced anemia, normocytic anemia secondary to bone marrow infiltration, and pancytopenia. We report a patient with SS, initially demonstrating widespread morbilliform eruption, who presented with malignancy-related microangiopathic hemolytic anemia (MAHA). Our findings represent a novel presentation of SS that will inform the differential diagnosis and treatment of future SS patients presenting with anemia and thrombocytopenia.
RESUMEN
BACKGROUND: Although treatment guidelines exist for melanoma in situ and invasive melanoma, guidelines for other melanocytic skin lesions do not exist. OBJECTIVE: To examine pathologists' treatment suggestions for a broad spectrum of melanocytic skin lesions and compare them with existing guidelines. METHODS: Pathologists (N = 187) completed a survey and then provided diagnoses and treatment suggestions for 240 melanocytic skin lesions. Physician characteristics associated with treatment suggestions were evaluated with multivariable modeling. RESULTS: Treatment suggestions were concordant with National Comprehensive Cancer Network guidelines for the majority of cases interpreted as melanoma in situ (73%) and invasive melanoma (86%). Greater variability of treatment suggestions was seen for other lesion types without existing treatment guidelines. Characteristics associated with provision of treatment suggestions discordant with National Comprehensive Cancer Network guidelines were low caseloads (invasive melanoma), lack of fellowship training or board certification (melanoma in situ), and more than 10 years of experience (invasive melanoma and melanoma in situ). LIMITATIONS: Pathologists could not perform immunohistochemical staining or other diagnostic tests; only 1 glass side was provided per biopsy case. CONCLUSIONS: Pathologists' treatment suggestions vary significantly for melanocytic lesions, with lower variability for lesion types with national guidelines. Results suggest the need for standardization of treatment guidelines for all melanocytic lesion types.
