Suspected coexistence of perianal necrotizing sweet syndrome in chronic myelomonocytic leukemia: A case report

Table 1 Diagnostic criteria for chronic myelomonocytic leukemia

Diagnostic criteria for CMML
Persistent PB monocytosis ≥ 1 × 109/L, with monocytes accounting for ≥ 10% of the WBC count
Not meeting WHO criteria for BCR-ABL1+ CML, PMF, PV, or ET
No evidence of PDGFRA, PDGFRB, or FGFR1 rearrangement or PCM1-JAK2 (should be specifically excluded in cases of eosinophilia)
< 20% blasts in the blood and BM
Dysplasia in 1 or more myeloid lineages. If myelodysplasia is absent or minimal, the diagnosis of CMML may still be made if the other requirements are met
An acquired clonal cytogenetic or molecular genetic abnormality is present in hemopoietic cells
The monocytosis (as previously defined) has persisted for at least 3 months
All other causes of monocytosis have been excluded

PB: Peripheral blood; WBC: White blood cells; BM: Bone marrow; WHO: World Health Organization; CMML: Chronic myelomonocytic leukemia; CML: Chronic myelogenous leukemia; PMF: Primary myelofibrosis, PV: Polycythemia vera; ET: Essential thrombocytosis.

Table 2 Major and minor criteria for Sweet’s syndrome

Major	(1) Abrupt onset of tender or painful erythematous plaques or nodules occasionally with vesicles, pustules or bullae
	(2) Predominantly neutrophilic infiltration in the dermis without leukocytoclastic vasculitis
Minor	(1) Preceded by a nonspecific respiratory or gastrointestinal tract infection or vaccination or associated with:
	Inflammatory diseases such as chronic autoimmune disorders, infections
	Hemoproliferative disorders or solid malignant tumors
	Pregnancy
	(2) Accompanied by periods of general malaise and fever (> 38 °C)
	(3) Laboratory values during onset: ESR > 20 mm; C-reactive protein positive; segmented-nuclear neutrophils and stabs > 70% in peripheral blood smear; leukocytosis > 8000 (three of four of these values necessary)
	(4) Excellent response to treatment with systemic corticosteroids or potassium iodide

Both major and two minor criteria are needed for diagnosis. ESR: Erythrocyte sedimentation rate.