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

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.

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

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.

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.