Furthermore, the disease may occur in association with the myelodysplastic syndrome or transform to acute myeloid leukemia. Consequently, BPDCN has a very low 5 year survival rate. Current translational research studies on treating BPDCN have therefore focused on non-chemotherapeutic regimens that target the molecular pathways which may promote the disease. Presentation
Blastic plasmacytoid dendritic cell neoplasm occurs in children, including neonates, but is more common in adults, particularly those between the ages 60–80. BPDCN usually (i.e. 61% to 90% of cases) presents with skin lesions, i.e. nodules, tumors, red or purple papules, bruise-like patches, and/or ulcers that most often occur on the head, face, and upper torso. The lesions are due to diffuse infiltrations of the skin by malignant pDC. In one large study, this presentation was accompanied by swollen lymph nodes, usually in the neck, due to malignant pDC infiltrations (~50% of cases); enlarged liver (~16% of cases) and/or spleen (26% of cases), also due to malignant pDC infiltrations; increased levels of malignant pDC in blood (i.e. >2% of nucleated cells) (~40% of cases), bone marrow (~65% of cases) and cerebrospinal fluid (47% of childhood cases but less often detected in adult cases). More advanced or severe cases may present with extreme organ and/or lymph node enlargements, skin lesions in virtually any site, and clinical evidence of malignant pDC infiltrations in the breasts, eyes, kidneys, lungs, gastrointestinal tract, bone, sinuses, ears, or testes. About 10% of individuals with BPDCN present with a leukemia-like disease, i.e.
);
tumor (e.g., laryngeal papillomatosis, squamous cell carcinoma of larynx, trachea or esophagus);
ALL (T-cell ALL can present with mediastinal mass that compresses the trachea and causes inspiratory stridor)

Diagnosis
Stridor is mainly diagnosed on the basis of history and physical examination, with a view to revealing the underlying problem or condition. Chest and neck x-rays, bronchoscopy, CT-scans, and/or MRIs may reveal structural pathology. Flexible fiberoptic bronchoscopy can also be very helpful, especially in assessing vocal cord function or in looking for signs of compression or infection. Treatments
The first issue of clinical concern in the setting of stridor is whether or not tracheal intubation or tracheostomy is immediately necessary. A reduction in oxygen saturation is considered a late sign of airway obstruction, particularly in a child with healthy lungs and normal gas exchange. Some patients will need immediate tracheal intubation. If intubation can be delayed for a period, a number of other potential options can be considered, depending on the severity of the situation and other clinical details. These include:

Expectant management with full monitoring, oxygen by face mask, and positioning the head on the bed for optimum conditions (e.g., 45 - 90 degrees). Use of nebulized racemic adrenaline epinephrine (0.5 to 0.75 ml of 2.25% racemic epinephrine added to 2.5 to 3 ml of normal saline) in cases where airway edema may be the cause of the stridor. (Nebulized Codeine in a dose not exceeding 3 mg/kg may also be used, but not together with racemic adrenaline [because of the risk of ventricular arrhythmias].)