Cutaneous-group histiocytoses associated with myeloid malignancies: A systematic review of 102 cases

Background: Histiocytoses are haematological disorders of bone marrow origin that share many biological and clinical features with haematological neoplasms. The association between histiocytoses of the cutaneous-group and myeloid malignancies is a poorly investigated topic of high biological and clinical impact.

Methods: We performed a systematic review of the scientific literature, compliant with PRISMA guidelines, to unravel the clinical and pathological features of this intriguing association.

Findings: We gathered and analysed 102 patients. Most were children with generalised cutaneous eruptions and displayed risk organ involvement (i.e. bone marrow, spleen, liver). Interestingly, all these features are uncommonly encountered in C-group histiocytosis not associated with haematological neoplasms.

Conclusions: Our review shows that generalised eruptions and risk organ involvement in cutaneous-group histiocytosis should raise a suspicion for a concomitant myeloid neoplasm both in children and in adults and warrant further investigations. A rapid recognition of this association is required to start a prompt and effective therapeutic management given the aggressive behaviour of the associated myeloid neoplasm in most instances.