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2016 ; 95
(15
): e3033
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Histiocytoid Sweet Syndrome Is More Frequently Associated With Myelodysplastic
Syndromes Than the Classical Neutrophilic Variant: A Comparative Series of 62
Patients
#MMPMID27082547
Ghoufi L
; Ortonne N
; Ingen-Housz-Oro S
; Barhoumi W
; Begon E
; Haioun C
; Pautas C
; Beckerich F
; Robin C
; Wolkenstein P
; Cordonnier C
; Chosidow O
; Toma A
Medicine (Baltimore)
2016[Apr]; 95
(15
): e3033
PMID27082547
show ga
Histiocytoid Sweet syndrome (H-SS) is a histological variant of Sweet syndrome
(SS) differing from classical neutrophilic SS (N-SS) by a dermal infiltrate
mainly composed of lymphocytes and histiocytoid myeloperoxidase-positive cells.
We aimed to report a large series of H-SS and compare the frequency and type of
hematological malignancies associated to H-SS and N-SS. We included 62 patients
with a coding histopathologic diagnosis of SS prospectively registered between
2005 and 2014 in the database of our Department of Pathology. Overall, 22 (35.5%)
and 40 (64.5%) patients had a histological diagnosis of H-SS and N-SS,
respectively. Median age, sex ratio, and cutaneous lesions were similar in the 2
groups. The frequency of extra-cutaneous manifestations was similar (50% vs
37.5%, P?=?0.42). Recurrent forms were significantly more frequent in H-SS than
in N-SS patients (21% vs 2.5%, P?=?0.01). A hematological malignancy was
diagnosed in 22 patients, 12 (55.5%) with H-SS and 10 (25%) with N-SS
(P?=?0.019). Hematological malignancy was of myeloid origin in 8/22 (36.3%) H-SS
and 5/40 (12.5%) N-SS patients (P?=?0.02), and of lymphoid origin without myeloid
component in 4/22 (18.1%) H-SS and 4/40 (10%) N-SS patients (P?=?0.35),
respectively. One N-SS patient had a hematological malignancy of mixed (myeloid
and lymphoid) phenotype. A myelodysplastic syndrome (MDS) was diagnosed in 7/22
(31.8%) H-SS and 1/40 (2.5%) N-SS patients (P?0.001). Hematological disease was
diagnosed before (in 8 H-SS and 3 N-SS patients) or at the time of the occurrence
of the cutaneous lesions (in 1 H-SS and 7 N-SS patients). However, in 3 H-SS
patients, all with MDS, cutaneous lesions preceded the hematological disease by
?6 months. In conclusion, H-SS was associated with MDS in one third of patients
but also with lymphoid malignancies, and cutaneous lesions could precede the
hematological diagnosis in patients with MDS. A complete hematological assessment
is mandatory at diagnosis, and monitoring blood cell counts should be recommended
for at least 6 months after the diagnosis of H-SS.