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Background  Lymphomatoid papulosis (LyP) can be associated with other haematological malignancies ( HM ), but reported percentages vary from 20% to over 50%.    Objective  To evaluate the frequency and prognostic significance of associated  HM  and non‐ HM  in LyP patients.    Methods  In this multicentre cohort study, the complete Dutch LyP population was included from the Dutch Cutaneous Lymphoma Registry between 1985 and 2018. Clinical and histopathological information was retrieved from every individual patient.    Results  After a median follow‐up of 120 months (range, 6–585), an associated  HM  was observed in 78/504 (15.5%) patients. Most common associated  HM  were mycosis fungoides ( MF ;  n  = 31) and anaplastic large‐cell lymphoma ( ALCL ;  n  = 29), while 19 patients had another  HM  of B‐cell ( n  = 14) or myeloid origin ( n  = 5). Even after a 25‐year follow‐up period, percentages of associated  HM  did not exceed 20%. Thirty‐nine of 465 patients (8.4%) without a prior or concurrent associated  HM  developed an associated  HM  during follow‐up, after a median of 68 months (range of 3–286 months). Nine of 78 patients died of associated  HM , including 6/22 patients developing extracutaneous  ALCL , while all patients with associated  MF  or skin‐limited  ALCL  had an excellent prognosis. Compared with the general population, LyP patients showed an increased risk (relative risk, 2.8; 95% confidence intervals, 2.4–3.3) for non‐ HM , in particular cutaneous squamous cell carcinoma, melanoma and intestinal/lung/bladder cancer.    Conclusions  An associated  HM  was reported in 15.5% of the LyP patients, particularly  MF  and  ALCL . Although the frequency of associated  HM  is lower than suggested and the prognosis of most patients with associated  HM  is excellent, a small subgroup will develop aggressive disease, in particular extracutaneous  ALCL . Furthermore, LyP patients have a higher risk of developing other malignancies. Clinicians should be aware of these risks, and LyP patients require close monitoring.

Frequency and prognosis of associated malignancies in 504 patients with lymphomatoid papulosis