Primary cutaneous follicle center lymphoma in a patient with <scp>WHIM</scp> syndrome

Yoshii, Yuta; Kato, Tamaki; Ono, Koji; Takahashi, Eishi; Fujimoto, Norihiro; Kobayashi, Shinichi; Kimura, Fumihiko; Nonoyama, Shigeaki; Satoh, Takahiro

doi:10.1111/jdv.12927

Cited by 12 publications

(6 citation statements)

References 7 publications

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“…3 Another case of PCFCL in the setting of the rare congenital immunodeficiency WHIM (warts, hypogammaglobulinemia, infections, and myelokathesis) syndrome has been described. 251 Another case was reported in a 16-year-old adolescent boy of PCFCL arising in the nose and extending into the maxillary sinus and soft palate. 252…”

Section: Lymphoproliferative Neoplasmsmentioning

confidence: 99%

Cutaneous Hematolymphoid and Histiocytic Proliferations in Children

Gru

Dehner

2018

Pediatr Dev Pathol

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This article focuses on cutaneous hematopoietic neoplasms that are more likely to be encountered in the pediatric age-group and includes both lymphoproliferative and histiocytic disorders. The cutaneous hematologic disorders in children have a different epidemiologic profile to what is seen during adulthood. Although mycosis fungoides is the most frequent form of cutaneous lymphoma in adults, it is very rare in children. Because lymphoblastic leukemias and lymphomas are more frequent in the pediatric setting, cutaneous leukemic infiltrates are relatively common in this age-group. Similarly, histiocytic disorders are more common in children, particularly Langerhans cell histiocytosis and juvenile xanthogranuloma. Notably, the histiocytic disorders have undergone significant modifications on their nomenclature in the basis of the molecular characteristics that are present in them. A summary of the most frequent cutaneous hematopoietic disorders in children will be discussed further in this review.

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Section: Lymphoproliferative Neoplasmsmentioning

confidence: 99%

Cutaneous Hematolymphoid and Histiocytic Proliferations in Children

Gru

Dehner

2018

Pediatr Dev Pathol

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“…Genital and oral HPV-related squamous cell carcinomas are also observed in patients with WHIM syndrome ( 35 , 36 ). Epstein–Barr virus (EBV) infections cause lymphoproliferative diseases and lymphoma, which are also observed in WHIM syndrome ( 34 , 37 ), and in cartilage–hair hypoplasia ( 32 ). Additionally, EBV causes EBV-smooth muscle tumors, which have been reported in liver, adrenals, and smooth muscle of patients with NK cell deficiencies ( 42 , 44 ).…”

Section: Resultsmentioning

confidence: 99%

A Tumor Profile in Primary Immune Deficiencies Challenges the Cancer Immune Surveillance Concept

Satgé

2018

Front. Immunol.

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Under the concept of cancer immune surveillance, individuals with primary immune deficiencies would be expected to develop many more malignancies and show an excess of all types of cancers, compared to people with a normal immune system. A review of the nine most frequent and best-documented human conditions with primary immune deficiency reveals a 1.6- to 2.3-fold global increase of cancer in the largest epidemiological studies. However, the spectrum of cancer types with higher frequencies is narrow, limited mainly to lymphoma, digestive tract cancers, and virus-induced cancers. Increased lymphoma is also reported in animal models of immune deficiency. Overstimulation of leukocytes, chronic inflammation, and viruses explain this tumor profile. This raises the question of cancers being foreign organisms or tissues. Organisms, such as bacteria, viruses, and parasites as well as non-compatible grafts are seen as foreign (non-self) and identified and destroyed or rejected by the body (self). As cancer cells rarely show strong (and unique) surface antibodies, their recognition and elimination by the immune system is theoretically questionable, challenging the immune surveillance concept. In the neonatal period, the immune system is weak, but spontaneous regression and good outcomes occur for some cancers, suggesting that non-immune factors are effective in controlling cancer. The idea of cancer as a group of cells that must be destroyed and eliminated appears instead as a legacy of methods and paradigms in microbiological medicine. As an alternative approach, cancer cells could be considered part of the body and could be controlled by an embryonic and neonatal environment.

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“…WHIM syndrome has characteristics of a PID that predispose to malignancies and hematological disorders . Lymphomas have been the only hematologic malignancies reported so far in WHIM syndrome (Table ). We report a female with WHIM syndrome who developed Hodgkin lymphoma (HL) and acute myeloid leukemia (AML) ensuing in less than 2 years.…”

Section: Lymphoma Cases In Whim Syndromementioning

confidence: 99%