How to treat myelodysplastic syndrome with clinical features resembling Behçet syndrome: a case-based systematic review

Yılmaz, Ümit; Ar, Muhlis Cem; Esatoğlu, Sinem Nihal; Bavunoğlu, İşıl; Erzın, Yusuf; Hatemi, Ali İbrahim; Yildirim, Suleyman; Hatemi, Gülen; Çelık, Aykut Ferhat

doi:10.1007/s00277-020-03951-5

Cited by 22 publications

(17 citation statements)

References 49 publications

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“…Therefore, it is necessary to carefully evaluate and control the disease status to the highest extent before allo-HSCT. However, BLD is often resistant to immunosuppressive therapy or corticosteroids [3] , and may not be adequately controlled before allo-HSCT. Taken together, careful and close monitoring is necessary at the time of allo-HSCT while considering the possibility that disease control before allo-HSCT may be inadequate and that bleeding from difficult-to-evaluate sites may occur.…”

Section: Discussionmentioning

confidence: 99%

“…However, an etiological association between MDS and BLD has not yet been demonstrated. Several reports have highlighted the potential efficacy of azacytidine [3] , but its clinical evaluation has yet to be determined. Allo-HSCT has been reported to control both MDS and BLD in some cases [3] , [4] , [5] , [6] , [7] , [8] .…”

Section: Introductionmentioning

confidence: 99%

“…Several reports have highlighted the potential efficacy of azacytidine [3] , but its clinical evaluation has yet to be determined. Allo-HSCT has been reported to control both MDS and BLD in some cases [3] , [4] , [5] , [6] , [7] , [8] . However, there have been limited reports of complications related to allo-HSCT for MDS with BLD.…”

Section: Introductionmentioning

confidence: 99%

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Successful allogeneic bone marrow transplantation after massive gastrointestinal bleeding in a patient with myelodysplastic syndrome associated with intestinal Behçet-like disease

Ishii

Tsukamoto

Mishina

et al. 2021

Leukemia Research Reports

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Successful allogeneic bone marrow transplantation after massive gastrointestinal bleeding in a patient with myelodysplastic syndrome associated with intestinal Behçet-like disease

Ishii

Tsukamoto

Mishina

et al. 2021

Leukemia Research Reports

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“…The IM in our small pediatric series, characterized by severe constitutional symptoms, slightly differs from the IM reported in adult's cohorts, where the common phenotypes were systemic vasculitis (32%), connective tissue disease (25%), arthritis (23%), and other symptoms. Constitutional symptoms were reported in up to 35% 30 . In the recently described VEXAS syndrome, most patients had recurrent fevers, pulmonary involvement, polychondritis, and dermatologic manifestations 16 .…”

Section: Discussionmentioning

confidence: 99%

“…19 Interestingly, no correlation has been established between response of IM and MDS activity. 20 MDS-IM in children is sparsely reported. 21,22 As most children with advanced MDS will undergo HSCT, reducing their IM before HSCT may dramatically improve their outcome.…”

Section: Introductionmentioning

confidence: 99%

Pediatric myelodysplastic syndrome with inflammatory manifestations: Diagnosis, genetics, treatment, and outcome

Yanir

Krauss

Stein

et al. 2021

Pediatric Blood & Cancer

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Background Inflammatory manifestations (IM) are well described in adult patients with myelodysplastic syndrome (MDS), but the presentation is highly variable and no standardized treatment exists. This phenomenon is rarely reported in children. As more pediatric patients are hematopoietic stem cell transplantation (HSCT) candidates, the role of anti‐inflammatory treatment in relation to HSCT should be defined. Procedure Here, we report a series of five children from a tertiary center. We describe the clinical presentation, molecular findings, and treatment options. Results All patients presented with advanced MDS with blast percentages ranging 10–30%, all had severe IM. One patient had MDS secondary to severe congenital neutropenia, the other four patients had presumably primary MDS. All four were found to harbor a PTPN11 gene driver mutation, which is found in 35% of cases of juvenile myelomonocytic leukemia (JMML). The mutation was present in the myeloid lineage but not in T lymphocytes. Three had symptoms of Behcet's‐like disease with trisomy 8 in their bone marrow. All patients were treated with anti‐inflammatory medications (mainly systemic steroids) in an attempt to bring them to allogeneic HSCT in a better clinical condition. All demonstrated clinical improvement as well as regression in their MDS status post anti‐inflammatory treatment. All have recovered from both MDS and their inflammatory symptoms post HSCT. Conclusion Primary pediatric MDS with IM is driven in some cases by PTPN11 mutations, and might be on the clinical spectrum of JMML. Anti‐inflammatory treatment may reverse MDS progression and improve the outcome of subsequent HSCT.

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The Skin and Disorders of the Haematopoietic and Immune Systems

Basu,

Kulasekararaj

2024

Rook's Textbook of Dermatology

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In this chapter the cutaneous manifestations of malignant and benign haematological disease are reviewed, as well as skin disorders associated with primary and acquired immunodeficiency. The vast array of skin manifestations associated with disorders of the haematopoietic and immune systems highlights the importance of close collaboration between dermatology, haematology and immunology teams to enable the delivery of optimal multidisciplinary patient care.

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How to treat myelodysplastic syndrome with clinical features resembling Behçet syndrome: a case-based systematic review

Cited by 22 publications

References 49 publications

Successful allogeneic bone marrow transplantation after massive gastrointestinal bleeding in a patient with myelodysplastic syndrome associated with intestinal Behçet-like disease

Successful allogeneic bone marrow transplantation after massive gastrointestinal bleeding in a patient with myelodysplastic syndrome associated with intestinal Behçet-like disease

Pediatric myelodysplastic syndrome with inflammatory manifestations: Diagnosis, genetics, treatment, and outcome

The Skin and Disorders of the Haematopoietic and Immune Systems

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