Blastic plasmacytoid dendritic cell neoplasm (BPDCN)
Journal Title: Αρχεία Ελληνικής Ιατρικής - Year 2011, Vol 28, Issue 5
Abstract
An 81-year-old man presented with one-month history of disseminated, painless, brownish, maculopapular or nodular skin lesions of the trunk and extremities. Initially, the patient presented with a large dark-brown plaque at the inner surface of the left ankle, with a maximum diameter of 4.5 cm. He was also complaining of weakness, fatigue and weight loss of 8 kg during the last 4 months (12% of body weight). His medical history was remarkable for arterial hypertension and “arrhythmia”, treated with oral amlodipine and carvedilol, respectively. Except of the skin lesions, physical examination revealed moderate splenomegaly (5 cm blcm), but no hepatomegaly. A complete blood count revealed normochromic-normocytic anemia, leukocytosis and thrombocytopenia, as follows: Hct 27.5%, RBC 2.96×1012/L, Hb 9.4 g/dL, MCV 92.6 fL, MCH 31.6 pg, MCHC 34.2 g/dL, WBC 17×109/L (differential count: 35% neutrophils, 18% lymphocytes, 49% blast cells) and PLTs 23×109/L (figures 1, 2). Serum biochemistry was rather unremarkable, except of mild hyperuricemia, with the following values: Urea 82 mg/dL, creatinine 1.1 mg/dL, total bilirubin 1.4 mg/dL, SGOT 41 U/L, SGPT 27 U/L, LDH 442 IU/L (upper normal limit 460 IU/L), uric acid 8 mg/dL and total proteins 6.71 g/dL with a normal electrophoretic diagram. Skin biopsies were performed from two separate lesions. Both biopsies revealed a diffuse dermal blast cell infiltrate. Immunohistochemical staining demonstrated that the neoplastic cells were positive for CD56, CD4 (+/-), CD123, TCL1, CD43 and terminal deoxynucleotidyl transferase (Tdt; few cells positive), while they were negative for CD3, CD20, CD79a, myeloperoxidase (MPO) and PGM1. A bone marrow aspiration and biopsy was performed. Bone marrow aspiration revealed extensive infiltration by medium-sized, monomorphous blast cells with irregular nuclei (Fig. 3). Blood and bone marrow immunophenotype confirmed and extended the findings of skin immunohistochemistry, as follows: CD56+, CD4 fully positive, CD123+, HLA-DR+, CD45RA+, 85k+ and Tdt+ (40% of cells). There was also positivity for CD7 and CD33 and weak positivity for CD117 and CD22, while CD3 and MPO were negative. Conventional cytogenetic analysis revealed a normal karyotype.
Authors and Affiliations
T. VASSILAKOPOULOS, E . ZOGRAFOS, L. PAPAGEORGIOU, V. KARALI, G. BOLUTSIKAS, K. PETEVI, TH. DALAGIORGOS, A. KANELLOPOULOS, P. FLEVARI, E. KOUTSI, Z. GALANI, M. ANGELOPOULOU, J. MELETIS
Keywords
Related Articles
- EP ID EP124482
- DOI -
- Views 58
- Downloads 0
Bathing as a means of therapy in the Byzantine Era
During the Byzantine Empire one of the best known methods of health care used as ancillary treatment for various illnesses was bathing. This form of treatment was adopted from ancient Greek medicine and applied in exactl...
Rendering small Journals viable and attractive
No abstract available
Ιστικές μικροσυστοιχίες στην ανοσοϊστοχημική και μοριακή διερεύνηση του συστήματος DNA MMR στον καρκίνο του παχέος εντέρου
ΣΚΟΠΟΣ Η εφαρμογή των ιστικών μικροσυστοιχιών και της υπολογιστικής μορφομετρίας στη συνδυασμένη διερεύνηση της ανοσοϊστοχημικής έκφρασης των πρωτεϊνών MLH1, MSH2 και MSH6 του συστήματος επιδιόρθωσης σφαλμάτων του DNA (D...
An audit of end-stage renal disease in a tertiary care hospital
OBJECTIVE To document the clinical profile of hemodialysis patients in a tertiary care hospital. METHOD The records of all patients hospitalized in the dialysis unit of the Alnoor Specialist Hospital, Makkah, Saudi Ar...
Antimalarial drugs <br /> <br />
In a recent World Health Organization report, malaria ranks fifth in terms of numbers of deaths due to infectious diseases, with almost one million deaths annually, mainly in Africa. Following a brief overview of the bio...