Abdominal complications of ascariasis in childhood.

OBJECTIVE: Complications of ascariasis are a significant cause of abdominal pain in pediatric emergencies, especially where it is endemic. A literature review was conducted with the aim of improving diagnostic and therapeutic approaches for these patients. DATA SOURCES: A PubMed search was conducted using the key terms "ascariasis complications" and "hepatobiliary ascariasis". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, case reports, and reviews published up to December 2023. SUMMARY OF FINDINGS: Obstruction of the small bowel is the most common complication. Others that are, rarer and more difficult to properly identify and treat, such as biliary, hepatic, and pancreatic complications, acute appendicitis, Meckel's diverticulitis, or peritoneal granulomas. Hepatic and pancreatic complications are rarer and more serious in children than in adults. While plain radiography is usually the only option in cases of intestinal obstruction, ultrasonography is the examination of choice in cases of hepatobiliary, pancreatic, and appendicular ascariasis complications in childhood. The treatment is clinical and conservative in most patients. Surgical treatment is indicated if conservative therapy fails, or if there are signs of complications. Laparoscopy has been used as an excellent technical alternative in adults with hepatobiliary complications of ascariasis, but further studies on its use in children are still needed. CONCLUSION: The creation of protocols and greater debate on this subject should be encouraged for a better understanding of the disease and to establish an early diagnosis and adequate treatment for children with complications resulting from massive infestation by Ascaris lumbricoides.

Circulating Cd34+ cell count differentiates primary myelofibrosis from other Philadelphia-negative myeloproliferative neoplasms: a pragmatic study.

A high number of circulating CD34+ cells has been advocated to distinguish primary myelofibrosis from other Philadelphia-negative myeloproliferative neoplasms. We re-evaluated the diagnostic interest of measuring circulating CD34+ cells in 26 healthy volunteers and 256 consecutive patients at diagnosis for whom a myeloproliferative neoplasm was suspected. The ROC curve analysis showed that a number of CD34+ <10/µl excludes the diagnosis of primary myelofibrosis with a sensitivity of 97 % and a specificity of 90 % (area under the curve: 0.93 [0.89-0.98]; p < 0.001). Patients with PMF harboring a CALR mutation had more circulating CD34+ cells than patients with either a JAK 2 or MPL mutation (p = 0.02 and p < 0.01, respectively). These results suggest that this fast, simple, non-invasive, and standardized test is of particular interest to exclude the diagnosis of primary myelofibrosis.

Prognostic impact of p27KIP1 expression in cyclin D1 positive lymphoproliferative disorders.

Nodal mantle cell lymphoma (MCL) is a well-defined entity, but non-nodal leukemic cyclin D1 positive lymphoproliferative disorders have been reported and their relationship with MCL remains controversial and their prognosis heterogeneous. We prospectively studied the expression of cyclin D1 in CD5 positive leukemic B lymphoproliferative disorders at diagnosis and identified 65 cases overexpressing cyclin D1. We did not distinguish any clinical or biological criteria allowing one to identify a non-MCL group. Multivariate analysis identified age, anemia and p27kip1 expression as independent prognostic factors of survival. By univariate analysis, p27kip1 high expression proved to be the strongest predictor of prolonged survival. The median survival of p27 low expressors was 30 months, while it was not reached for p27 high expressors. A high level of p27 expression was often found associated with the absence of nodal involvement and the presence of somatic mutations, but neither of them was restricted to the p27 high expression group. In conclusion, we hypothesize that MCL and these cyclin D1 positive leukemic lymphoproliferative disorders represent a continuous spectrum of diseases. Determination of p27 expression level appears as a routine applicable test allowing identification of a subset of patients who could be considered for different therapeutic approaches.

Cyclin D1 overexpression allows identification of an aggressive subset of leukemic lymphoproliferative disorder.

The conjunction of clinical features, cell morphology and immunological characteristics allows an accurate diagnosis in most cases of B cell chronic lymphoproliferative disorders (CLD). However, the diagnosis remains uncertain in a small percentage of cases, often referred as to unclassified B cell proliferation or atypical chronic lymphocytic leukemia (CLL). We have studied retrospectively the 192 cases of leukemic CLD seen in our institution over a 3-year period, for which both clinical and routine biological data at presentation were available. Forty cases (20%) did not fit into any of the well-identified categories according to the FAB criteria and remained unclassified. We assessed cyclin D1 expression in all of these cases and found that 10 of them expressed a high level of cyclin D1 protein. We compared the characteristics of these 10 cases with those of the 30 cyclin D1 negative CLD. Despite non-distinctive cytological and phenotypic features, the 10 cyclin D1 positive patients exhibited a strikingly uniform clinical presentation with elevated leukocytosis, massive spleen enlargement and no superficial lymphadenopathy. Their outcome was very poor with a median survival of 10 months, contrasting with the prolonged survival of the cyclin D1 negative patients. The cytological features of tumor cells from these 10 patients with cyclin D1 positive unclassified leukemic CLD were similar to those of the circulating lymphoid cells from 15 patients with histologically proven mantle cell lymphoma (MCL) and primary or secondary blood involvement. Therefore, cyclin D1 expression allowed identification among the unclassified CLD, a subset of aggressive disorders which represent a leukemic counterpart of MCL (mantle cell leukemia). We suggest that determination of cyclin D1 expression by any technique available should be systematically included when investigating atypical CLL.

[Jaundice with free bilirubin following preventive anti-retroviral therapy after sexual aggression]. / Ictère à bilirubine libre au cours d'un traitement prophylactique antirétroviral après agression sexuelle.

BACKGROUND: Interest has recently focused on anti-HIV prophylaxis in case of sexual exposure. A circular from the French Ministry of Health (DGS/DH n(o) 97/560, 12 August 1997) envisages such treatment in certain risk situations such as sexual aggression. The toxic risk of prescribing a tritherapy or a bitherapy, even for a short period of a few weeks must be considered. CASE REPORT: A 20-year-old rape victim with an uneventful medical history was given a prophylactic regimen including zidovudine, laminovudine and indinavir. Three months later, she developed free-bilirubin jaundice with biological signs of hemolysis. DISCUSSION: We draw attention to the risk of severe adverse effects of short-duration anti-HIV prophylaxis in apparently healthy subjects. The protocol must included careful patient information and rigorous surveillance.

[Myelodysplasia and tuberculosis]. / Myélodysplasie et tuberculose.

We report here the case of a patient with myelodysplasia and concomitant tuberculosis. Refractory anemia with blasts excess diagnosis was based upon morphological and cytogenetical criterias (del 20q), and tuberculosis was diagnosed on a cervical lymph node biopsy. Hematological data remained stable without any specific treatment for several months, cell counts even normalized under antituberculosis tritherapy. Clinical and hematological worsening appeared 3 years later, 1 year after discontinuation of antituberculosis therapy. It was characterized by progressive bone marrow failure and transformation in acute myeloid leukaemia. Concomitantly tuberculosis relapsed. The association of antituberculosis therapy and polychemotherapy (daunorubicine and aracytine) did not allow to obtain a hematological remission. The relationship between tuberculosis and myelodysplasia is discussed.