Sweet’s syndrome associated with clonal hematopoiesis of indeterminate potential responsive to 5-azacitidine

Abstract

Sweet's syndrome (SS) is a rare condition characterized by the abrupt appearance of painful skin lesions due to neutrophilic dermal infiltration. Hematologic neoplasms, particularly acute myeloid leukemia (AML) and myelodysplastic syndromes (MDSs), have been commonly reported in association with SS. Clonal hematopoiesis of indeterminate potential (CHIP) is an emerging entity that is a precursor state to myeloid neoplasms. CHIP has not been previously associated with SS. We report the case of a 71-year-old man who presented with recurrent, painful edematous and erythematous papules and nodules for 18 months despite treatment with corticosteroids. He had normal blood counts, but a macrocytosis was noted (110 fl). Alternative causes of macrocytosis were ruled out. A skin biopsy confirmed a diagnosis of SS. Bone marrow biopsy specimen yielded a normal karyotype except for loss of the Y chromosome and equivocal morphologic findings. Polymerase chain reaction (PCR) and reverse transcription polymerase chain reaction (RT-PCR) of selected genes from the peripheral blood demonstrated a mixed lineage leukemia (MLL) partial tandem duplication (PTD) and sequence variant in CCAAT/ enhancer binding protein alpha (CEBPA). These findings were consistent with a diagnosis of CHIP. The patient was treated with 5-azacitidine and achieved a complete remission of his skin lesions and was able to discontinue corticosteroids. To our knowledge, this is the first report of a patient with recurrent SS associated with CHIP. In addition to other myeloid neoplasms like AML and MDS, CHIP should be considered as a potential etiology in cases of recurrent SS. Treatment with a hypomethylating agents such as azacitidine could also serve as an alternative to systemic corticosteroid therapy.

IDH2-mutated near ETP-ALL with aggressive leukemia cutis and brisk response to venetoclax and decitabine

“…Moreover, as data on CH and myeloid mutations in lymphoid malignancies is just recently emerging, there is no evidence to support a clear association between the development of cutaneous lesions in T-ALL and pre-existing CHIP. It has been previously reported that a case of Sweet's Syndrome (SS), a neutrophilic dermatosis often found in hematologic malignancies, which occurred in a patient with CHIP responded to the HMA 5-azacitidine [21] . Although SS is pathologically distinct from leukemia cutis, it is of interest to determine if dermatologic manifestations associated underlying CH are more responsive to HMA-based regimens.…”

Atypical presentation of Sweet syndrome with nodular erythema and oral ulcerations provoked by Ad26.COV2.SSARS‐CoV‐2 vaccination and review of literature

“… 28 Among the most commonly described causes of malignancy‐associated SS are hematologic neoplasms like acute myeloid leukemia and myelodysplastic syndrome, but also large B‐cell lymphoma, Hodgkin lymphoma, and others like clonal hematopoiesis of indeterminate potential and solid tumors of a gastrointestinal tract, breast, or testicular carcinoma. 14 , 29 In some cases SS precedes the malignancy, thus acts as a paraneoplastic syndrome. The medications causing the onset of the disease are very often anti‐cancer agents: granulocyte‐colony stimulating factor (G‐CSF), bortezomib, azacitidine, decitabine, but also other drugs—antibiotics: minocycline, trimethoprim‐sulfamethoxazole, antiepileptics: carbamazepine, diazepam, and others.…”

“…It may be also associated with common variable immunodeficiency (CVID) 28 . Among the most commonly described causes of malignancy‐associated SS are hematologic neoplasms like acute myeloid leukemia and myelodysplastic syndrome, but also large B‐cell lymphoma, Hodgkin lymphoma, and others like clonal hematopoiesis of indeterminate potential and solid tumors of a gastrointestinal tract, breast, or testicular carcinoma 14,29 . In some cases SS precedes the malignancy, thus acts as a paraneoplastic syndrome.…”