pancytopenia. The patient was given high-dose cytosine arabinoside (ara-C) chemotherapy and attained clinical re- mission. Unfortunately the disease relapsed again in April 2011 with multiple new skin lesions and 80% blasts in the peripheral blood. Due to resistant/refractory to treatment, comfort and supportive measures were given to the patient. The patient died of the disease 17 months after diagnosis. DISCUSSION Clinical Features Since BPDCNwas classified as a distinct entity byWHO in 2008, more than 200 cases that meet the new diagnostic criteria have been reported in the literature. 4 BPDCN is a rare hematopoietic malignancy without known predispos- ing factors. It has a male-to-female ratio of 2.6-2.7:1. 3,7,8 Most patients are elderly with a median age of 62-67 years at the time of diagnosis. 3,7-9 Pediatric cases as young as 4 years of age 10 and a unique congenital case of a 3-day-old baby (Yang et al., 2012), although much less common, have been reported. The diagnosis is generally based on skin biopsy. BPDCNusually presents with solitary, multiple, or general- ized skin or subcutaneous lesions in the presence or absence of systemic involvement such as peripheral blood, bone marrow, and lymph nodes. 4 The skin lesions can bemacules, papules, plaques, or nodules and range in size from 0.5 to 12 cmwith a median size of 3 cm at diagnosis. 8,10 Ulceration is not a feature, although Jegalian et al. reported a 12-year-old female patient presenting with a 12 cm ulcerated cutaneous lesion died from infectious complication six months after diagnosis. 10 In the largest case series since 2008 by Dalle et al., all 47 patients had cutaneous involvement (100%) at diagnosis, with 40% as solitary lesion, 38% as multiple le- sions affecting one or two areas, and 21% as disseminated skin disease. 7 Systemic involvement at diagnosis usually occurs in bone marrow (48%-68%), peripheral blood (33%- 73%), and lymph nodes (24%-41%). 7-9 Initial presentation at uncommon sites including maxilla, breast, kidney, liver, spleen, and lung has been reported in very rare cases. 10,11 To the best of our knowledge, our patient is the first case presentingwith CNS involvement since the latestWHO clas- sification in 2008. By literature studies, no cases with CNS involvement at diagnosis were reported, although relapse in the CNS was documented in few cases. 12,13 Nearly half of BPDCN patients (35%-48%) presented with only cutaneous disease at diagnosis. 7-9 Nonetheless, systemic involvement eventually develops with progression of the disease. Leuke- mic disease without cutaneous involvement at presentation, albeit rare, has been reported. 8,12,14 Although response occurs after treatment, relapse invariably occurs, and fatal outcome occurs rapidly. Complete remission (CR) after initial treat- ment was seen in 47%-68%patients with amean relapse-free period of 12.6 months (range 2-42 months). 7-9 BPDCN has an aggressive course and a poor prognosis with a mean survival of 16.7 months. 7 In an Austrian study of 33 patients, there was no difference in survival between patients with
cutaneous disease only and patients with both cutaneous and extracutaneous diseases. 15 Initial staging results did not affect survival. 7,15 Therefore, even though BPDCN presents with only cutaneous diseases in almost half of the cases, it has been suggested these patients should also be treated with initial aggressive therapy. Treatments Due to its rarity and dramatically evolving recognition, no standard treatment is available, and patients usually receive multiple regimens. First-line treatments are vari- able, including palliative care, monochemotherapy, non- Hodgkin’s lymphoma (NHL)-type, acute myeloid leukemia (AML)-type and acute lymphoblastic lymphoma (ALL)- type, radiation therapy, and BMT/SCT. Previous studies on formerly called blastic NK-cell lymphoma/agranular CD4+CD56+ hematodermic neoplasm demonstrated that CHOP/CHOP-like regimen was inadequate; AML-type regimen was associated with a poor prognosis; and hyper- CVAD regimenwas associatedwith a favorable prognosis. 16 Dalle et al. showed that, when used as first-line therapy, CHOP/CHOP-like regimens and radiation therapy had a mean relapse free survival of five and five and a half months respectively compared to 25.3 months with BMT. 7 In a re- cent retrospective study of 39 BPDCN patients (Roos-Weil et al., 2013), allogeneic SCT in first remission was associ- ated with favorable survival, while age, donor type, stem cell source, and chronic graft versus host disease (GVHD) had no significant impact on outcomes. In a case series of six BPDCN patients (Dietrich et al., 2010), two patients without allogeneic SCT died rapidly of the disease at six and 13 months after first-line treatment; two patients with allogeneic SCT in active disease achieved CR but relapsed at six and 18 months after transplantation; and two patients with allogeneic SCT in remission remained disease free at 57 and 16 months after SCT. The authors suggested that allogeneic SCT in first CR should be considered in patients up to 70 years of age. Dalle et al. showed that survival of patients with BMT was significantly higher (31.3 months vs. 12.8 months) than those without BMT. 7 Ham et al. (2012) reported a 26-year-old patient who was treated with AML- type chemotherapy followed by allogeneic SCT in first CR-achieved long-term disease free survival at 30 months after diagnosis. Jegalian et al. indicated that BPDCN was less aggressive in pediatric patients than in adult patients. Of the total 25 pediatric patients, all three patients who received AML-type therapy were dead at the time of clini- cal follow-up. In contrast, among 14 patients who received ALL-type therapy, only one patient who presented with a 12 cm ulcerated cutaneous lesion died of therapy related infectious complications. The authors suggested that, for pediatric patients, high-riskALL-type chemotherapy should be used as first-line treatment, and SCTmay be reserved for those with relapse of disease or on their second remission. 10 Interestingly, Dohm et al. (2011) reported a patient after allogeneic SCT developed fulminant leukemic progression
J La State Med Soc VOL 166 January/February 2014 5
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