Primary cutaneous immunocytoma/marginal zone B-cell lymphoma: a case with unusual course

Although primary cutaneous immunocytoma/marginal zone B-cell lymphoma (PCI/MZBL) is considered as an indolent lymphoma with excellent prognosis, extracutaneous spread and even death due to disseminated disease have been reported. This is a case report of a PCI/MZBL showing dissemination to the regional lymph node and bone marrow, 22 months after the initial diagnosis. Furthermore, in the lymph node, there were focal areas of high-grade transformation. On the other hand, the bone marrow involvement displayed low-grade morphology. We suggest that it should be kept in mind that there is still a possibility of systemic involvement in PCI/MZBL.     

Late cardiac evaluation of children with solid tumors after anthracycline chemotherapy

BACKGROUND: The therapeutic potential of anthracycline antibiotics is limited by their cardiotoxicity. Electrocardiography, exercise testing, and two-dimensional echocardiography are non-invasive techniques used in the follow-up of children for cardiotoxicity. Plasma B-type natriuretic peptide (BNP) levels are thought to be useful markers in the early detection of AC induced cardiomyopathy. PROCEDURE: We evaluated cardiac status of 34 patients with solid tumors treated with anthracycline antibiotics. All of the patients were asymptomatic and had no evidence of residual malignancy. They were evaluated by electrocardiography, exercise testing, echocardiography, and plasma BNP levels measured before and after the exercise testing. RESULTS: Electrocardiography revealed only minor abnormalities of little clinical significance. All of the patients completed the exercise testing without complication, and the duration of the exercise for each patient was between normal limits. Cardiac output (CO) and wall stress (WS) were significantly increased in patients, than in controls in echocardiographic evaluation of systolic functions (P < 0.001). Diastolic filling patterns showed various abnormalities; M-E, M-A, T-E, T-A, AT, and IVRT were significantly higher than those of controls. Mean plasma BNP levels of the patients (10.56 +/- 10.22 pg/ml) were significantly higher than BNP levels of the healthy controls (4.09 +/- 2.26 pg/ml) (P < 0.016), before exercise testing. The mean plasma BNP levels of the patients (15.70 +/- 14.06 pg/ml) were higher than resting state after exercise testing, but it was not statistically significant (P > 0.05). CONCLUSION: Our findings demonstrated that echocardiographic and biochemical abnormalities could be found even at low cumulative doses of AC antibiotics. The use of serial echocardiographic studies and plasma BNP determinations to identify high-risk patients for cardiotoxicity needs to be verified by additional studies.     

Randomised unicenter trial for comparison of three regimens inde novo adult acute nonlymphoblastic leukaemia

Various regimens have been explored in the treatment of acute nonlymphoblastic leukaemia (AML), but so far none has been shown to be superior. Here we report on a comparison of three widely used protocols defined by Berman (Group 1), MRC AML 10 (Group 2), and Arlin (Group 3). Group 1 includes cytosine arabinoside (Ara-C) (100 mg/m²/d, days 1–7) and idarubicin (Ida) (12 mg/m²/d, days 1–3) for induction, and Ara-C (200 mg/m²/d, days 1–6) and Ida (15 mg/m²/d, day 1) twice for consolidation. Group 2 includes Ara-C (200 mg/m²/d, days 1–10), daunorubicin (Dnc) (50 mg/m²/d, days 1, 3, 5) and etoposide (VP16) (100 mg/m²/d, days 1–5) for induction. The first consolidation therapy consisted of the same schedule except for Ara-C given on days 1–8. The second consolidation regimen consisted of Ara-C (200 mg/m²/d, days 1–8), VP16 (100 mg/m²/d, days 1–5) and amsacrine (100 mg/m²/d, days 1–5). Mitoxantrone (Mitox) (10 mg/m²/d, days 1–5) and Ara-C (200 mg/m²/d, days 1–3) were given as the third consolidation therapy. Group 3 was identical to Group 1 except for Ida being replaced with Mitox. During the study period 99 patients were enrolled and 34 were allocated randomly to Group 1, 36 to Group 2, and 29 to Group 3. Except for age distribution all patients’ characteristics were similar between the groups. As there were more elderly patients in Group 1, time to complete remission (CR) was longer in this group as they needed more second induction. Induction deaths were 9.7%, 12.9% and 14.8% in Groups 1, 2 and 3, respectively. Patients in Group 2 received a higher amount of Ara-C compared with the other groups (P<0.001). After a median follow-up period of 45 months (1–67 for survivors) an advantage in Group 1 was observed. Relapse-free survival (RFS) was better in Group 1 (P=0.014) at 3 years. Fourteen of the patients were transplanted (11 allografts, 3 autografts). When patients with transplants were excluded, overall survival was longer in Group 1 both at 3 years and 5 years (P=0.05). In conclusion, despite patient advanced age and lower dose of Ara-C, the idarubicin-containing treatment was superior to the other regimens.     

Efficacy,safety and drug survival of conventional agents in pediatric psoriasis: A multicenter,cohort study

The data on long‐term efficacy, safety and drug survival rates of conventional systemic therapeutics in pediatric psoriasis is lacking. The primary aim of this study is to investigate acitretin, methotrexate, cyclosporin efficacy, safety and drug survival rates in pediatric patients as well as predictors of drug survival. This is a multicenter study including 289 pediatric cases being treated with acitretin, methotrexate and cyclosporin in four academic referral centers. Efficacy, adverse events, reasons for discontinuation, 1, 2‐ and 3‐year drug survival rates, and determinants of drug survival were analyzed. A 75% reduction of Psoriasis Area and Severity Index score or better response rate was obtained in 47.5%, 34.1% and 40% of the patients who were treated with acitretin, methotrexate and cyclosporin, respectively. One‐year drug survival rates for acitretin, methotrexate and cyclosporin were 36.3%, 21.1% and 15.1%, respectively. The most significant determinant of drug survival, which diminished over time, was treatment response whereas arthritis, body mass index and sex had no influence. Although all three medications are effective and relatively safe in children, drug survival rates are low due to safety concerns at this age group. Effective disease control through their rational use can be expected to improve survival rates.     

Effectiveness of medium-dose ultraviolet A1 phototherapy in localized scleroderma

Background Recently, ultraviolet (UV) A1 phototherapy has been suggested as an effective treatment for localized scleroderma (LS); however, the optimal dose of UVA1 still has not been determined. Objective We aimed to evaluate the therapeutic effectiveness of medium‐dose (30 J/cm²) UVA1 phototherapy and to show that 13 MHz ultrasound is a valuable tool for assessing the results of UVA1 phototherapy in LS. Methods Thirty‐five patients with LS were treated with medium‐dose (30 J/cm²) UVA1. In total, 30–45 treatments and 900–1350 J/cm² cumulative UVA1 doses were evaluated by clinical scoring in all patients. In 14 patients, skin thickness was also determined by 13 MHz ultrasound examination. Results In all patients, medium‐dose UVA1 therapy softened sclerotic plaques, and marked clinical improvement was observed in 29 of 35 (82. 85%) patients. Ultrasound measurements showed that skin thickness was significantly reduced. No side effects were observed during or after treatment. Conclusion Medium‐dose UVA1 phototherapy is a highly effective, safe, and well‐tolerated therapeutic modality for treatment of all types of LS. A 13 MHz ultrasound probe may be used for evaluating UVA1 phototherapy results.     

Dermoscopic Evolution of Pediatric Nevi

BackgroundThe incidence of pediatric melanoma is very rare. Dermoscopic features help to distinguish pediatric melanoma and common nevi.ObjectiveTo study the evolution of dermoscopic findings in benign nevi in childhood through serial observation and photography.MethodsWe examined 504 melanocytic lesions in 100 patients. From each participant, dermoscopic images of the nevi from 4-year dermoscopic follow-up were obtained, including randomly selected nevi.ResultsThe most common dermoscopic patterns were homogeneous (193 nevi; 38.3%), globular (92 nevi; 18.3%), and reticular (86 nevi; 17.1%). Dermoscopic pattern changes were detected in 27% of patients aged 2~10 years and in 20% of patients aged 11~16 years. The main pattern changes consisted of the transition from homogeneous to globular-homogeneous (16%), from homogeneous to reticular-homogeneous (12%) and from globular to globular-homogeneous (10%). Although 257 of the 504 nevi (51.0%) have stable duration without size changes, 169 of the 504 nevi (33.5%) were enlarged, and 78 of the 504 nevi (15.5%) had become smaller.ConclusionThese results contrast with the prevailing view that dermoscopic patterns in pediatric nevi are usually characterized by globular patterns and that melanocytic nevi generally undergo a characteristic transition from a globular pattern to a reticular pattern. Fifty one percent of patients did not exhibit a size change. While 33% of patients had symmetrical enlargement, 15% of patients had involution. Therefore, enlargement is a common dermoscopic change in pediatric nevi, and is not a specific sign of pediatric melanoma.     

Three years dermoscopic follow‐up of atypical nevi

Atypical nevi are dynamic lesions and may progressively transform into more or less atypical lesions. We aimed to investigate the dermoscopic features of atypical nevi and dynamic changes in these lesions over a period of 3‐years. Patients with 3‐year dermoscopic follow‐up records were enrolled in the study. We compared the dermatoscopic features of the nevus recorded in the first dermoscopic examination and at the end of the third year. Changes in size (mm), pattern, and color were investigated. The most common dermoscopic patterns were reticular (18 patients; 34%), reticular‐homogeneous (17 patients; 32.1%), and reticular‐globular (7 patients; 13.2%). The most common pigmentation patterns were central hyperpigmentation (28 patients; 52.8%), regular pigmentation (nine patients; 17.0%), and multifocal hypo/hyperpigmentation (eight patients; 15.1%). Twenty‐one (39.6%) patients showed changes in pattern. The transformation from reticular‐homogeneous pattern to the homogeneous pattern was the most frequent change in pattern (7 of 21 patients; 33.3%). The transformation from reticular pattern to reticular‐homogeneous pattern was the second most common change in pattern (5 of 21 patients; 23.8%). Fourteen (26.4%) patients experienced symmetrical enlargement. Symmetrical enlargement was statistically more frequent in patients who showed dermoscopic changes in pattern than in those who did not show any changes in the pattern (p: .038). In this study, we did not observe any new dermoscopic clues for the diagnosis of melanoma during the follow‐up. The nevi tended to turn into a homogeneous (structureless) pattern. We observed that the most common dermoscopic change in pattern was the transformation from reticular‐homogeneous pattern to homogeneous pattern, and the lesions had symmetrical enlargement during this transformation. In conclusion, despite the known association between atypical nevi and the risk of developing melanoma, most atypical nevi do not transform into melanoma. Therefore, our study suggests that the excision of atypical nevi is not necessary and dermatoscopic follow‐up can reduce the number of unnecessary excisions.     

Echogenic focus in the fetal left ventricular cavity: Is it a false tendon?

Objective

To draw attention to the left ventricular false tendon which can be misinterpreted as echogenic focus in the fetus.

Methods

The study group consisted of 9 fetuses out of the 161 who had been misdiagnosed for left ventricular false tendon as echogenic focus by obstetricians. Fetal echocardiography and 2-D color Doppler echocardiography were performed in the pre–postnatal period. The standard fetal echocardiographic views (4,5 chamber views, long axis view of the left ventricle, short axis view of the ventricles and great arteries, three vessels and trachea view, long axis views of the duct and aortic arch) were obtained for each case.

Results

Of the 161 fetuses with echogenic focus in the left ventricle which underwent fetal echocardiography, 9 (5.6%) were diagnosed with false tendons present in the left ventricular cavity with no other cardiovascular anomaly. Six out of 9 patients underwent amniocentesis as follows: for age of over 35 years (two patients), abnormal double-triple screening tests plus echogenic focus (two patients) and soft ultrasonographic markers including echogenic focus (two patients). These fetuses revealed no cardiovascular and other systemic pathology or dysmorphism except for false tendons in the left ventricular cavity.

Conclusion

False tendon should be taken into account as differential diagnosis of left ventricular echogenic focus in the fetus. Misinterpretation of false tendon as echogenic focus may cause unnecessary fetal invasive approach and maternal anxiety, especially when it arises with a background of borderline fetal findings and knowledge.