[T-cell prolymphocytic leukemia: potential diagnostic pitfalls]. / Leucémie prolymphocytaire T : un piège diagnostique à connaître.

BACKGROUND: Sezary syndrome (SS) presents clinically as erythroderma, which may be pigmented, and pruritic, associated with peripheral lymphadenopathies. Erythroderma may also occur in a broad range of reactive and malignant conditions including T-cell prolymphocytic leukemia (T-PLL). We report a case initially diagnosed as SS but ultimately diagnosed as T-PLL based upon skin involvement. CASE REPORT: A 70-year-old man was referred by his hematologist for management of SS. Physical examination revealed lymphadenopathies and mild diffuse erythema without infiltration. His WBC count was elevated at 8.3 G/L. A peripheral blood smear showed Sezary-like cells. Flow cytometry of peripheral blood revealed prolymphocytic T-cells staining positively for CD2, CD3, CD4 and CD7. Cytogenetic studies showed chromosomal abnormalities in terms of number and structure with missing chromosomes 6 and13, as well as deletion of chromosome 17. Finally, a diagnosis of T-PLL was made. Pentostatin was initiated pending treatment with alemtuzumab, but the patient's overall condition deteriorated rapidly and he died 10 days later. DISCUSSION: Diagnosis of LPLT is based upon a number of factors. In the case presented herein, the clinically atypical nature of the skin lesions prompted the dermatologist to review the diagnosis. The morphology of the circulating T-lymphocytes and their immunologic and phenotypic characteristics finally ruled out the diagnosis of Sezary syndrome, while their association with compatible cytogenetic anomalies enabled a diagnosis of prolymphocytic leukemia to be made instead. CONCLUSION: Prolymphocytic leukemia involves complex differential diagnosis with regard to Sezary syndrome, posing potential pitfalls for hematologists and dermatologists.

[Yellow nail syndrome: two pediatric case reports]. / Syndrome des ongles jaunes : deux cas pédiatriques.

BACKGROUND: Yellow nail syndrome (YNS) is a combination of specific nail dystrophy (yellowish or green nails, transverse ridging, increased curvature with "a hump" and distal onycholysis), lymphoedema and respiratory tract involvement (mainly bronchial hyper-responsiveness, recurrent pneumonia, bronchiectasis and pleural effusion), with or without sinusitis. This rare condition is most often seen in middle age. We report two pediatric cases. PATIENTS: A 9-year-old boy had 20 yellow nails and lymphedema of the lower and upper left limbs. He was born at 32 weeks of gestation. At birth, he had fetal hydrops with bilateral chylothorax. Since then, he presented bronchopulmonary dysplasia. In his first year of life, he had recurrent nasopharyngitis and serosal otitis. Lymphoscintigraphy was not performed. A 14-year-old boy had 10 yellow toenails and 2 yellow fingernails. He was born at 36 weeks of gestation with fetal hydrops. He had recurrent serosal otitis and asthma attacks. Lymphoscintigraphy showed lymphatic hypoplasia, especially of the left limbs. DISCUSSION: These two children have the classic triad of YNS, including yellow nails, lymphedema and respiratory tract involvement. Thirteen cases have been reported in children from birth to 10 years of age, three of whom were siblings, thus suggesting genetic predisposition. In most cases lymphoscintigraphy showed hypoplasia and/or ectasia of lymphatic vessels. These lymphatic abnormalities may be responsible for nail changes as seen in Turner's syndrome. The prognosis of YNS in adults may be serious because of the pulmonary tract involvement. However, it is not known in children. CONCLUSION: Yellow dystrophic nails in children may be associated with lymphedema and respiratory tract involvement, as in adults, and this condition should therefore be investigated in cases of YNS.

[Acute obstructive renal failure and scrotal complete hernia of the bladder. Apropos of a case, review of the literature]. / Insuffisance rénale aiguë obstructive et hernie scrotale complète de la vessie. A propos d'une observation, revue de la littérature.

Massive herniation of the bladder into the scrotum is uncommon and can have a deleterious effect on the upper urinary tract. We report a case where complete scrotal hernia of the bladder lead to obstructive renal failure due to torsion of the trigone. Most simple bladder hernias can be explained by a prostate or uretral obstacle. The mechanism in our patient with no known prostate or uretral disease was the passage of the bladder via a defective inguinal canal caused by excessive obesity. This type of massive hernia has been reported in Sumo boxers. The effect on the upper urinary tract may be related to a fold in the terminal ureter or more exceptionally in the trigone. Immediate treatment requires urine drainage above the obstacle and intensive care. After stabilization, the anatomic position of the excluded bladder is re-established with reconstruction of the inguinal area, usually with a prosthesis. Any obstacle below the bladder must be removed.