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

Abstract

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 cutaneousgroup 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.

MAP2K1‐driven mixed Langerhans cell histiocytosis, Rosai‐Dorfman‐Destombes disease and Erdheim‐Chester disease, clonally related to acute myeloid leukemia

“…MH seems to bear a higher frequency of BRAF mutation in comparison to LCH or ECD 5,6 . Histiocytoses‐AHN are an emerging topic in hematology‐oncology 7,8 . C‐group histiocytoses‐AHN are more frequently diagnosed in male children, with MS pictures, risk‐organ involvement, and poor overall survival.…”

“…Overall, 71.6% of patients have risk‐organ involvement (ie, spleen, liver, or hematopoietic system). To date the most frequently diagnosed histiocytosis‐AHN is juvenile or adult xanthogranuloma 8 . Myeloid neoplasms outnumber lymphoid malignancies in both L‐ and C‐group histiocytosis‐AHN patients, whereas histiocytic sarcoma (M‐group) patients more often develop low‐grade B‐cell lymphoma or T‐acute lymphoblastic leukemia 8,9 .…”

“…To date the most frequently diagnosed histiocytosis‐AHN is juvenile or adult xanthogranuloma 8 . Myeloid neoplasms outnumber lymphoid malignancies in both L‐ and C‐group histiocytosis‐AHN patients, whereas histiocytic sarcoma (M‐group) patients more often develop low‐grade B‐cell lymphoma or T‐acute lymphoblastic leukemia 8,9 . In C‐group histiocytoses‐AHN, 71% of the AHN was of mononuclear‐phagocyte lineage (ie, juvenile or chronic myelomonocytic leukemia, acute monocytic or myelomonocytic leukemia), the same as histiocytoses 8 …”

“…Hematological (and especially myeloid) neoplasms may arise in up to 10% of adult histiocytosis patients, with a higher frequency comparing to the general population 7 and eventually share clonal alteration(s) with histiocytosis. Such association correlate with a poor patients' prognosis as well as difficulties in the management 7‐9 …”

Pearls and updates: cutaneous signs of systemic malignancy

“…SS-LCH may develop into MS-LCH years after initial remission and there are currently no histologic or molecular markers that distinguish which SS-LCH cases will progress, thus both diagnoses require long-term follow-up [35 ▪▪ ,37,42]. A 2021 report analyzing 102 cases suggested an association of LCH with myelogenous malignancies further necessitating regular surveillance [40 ▪ ].…”