Normal values for mean platelet volume (MPV) and platelet distribution width (PDW) have not been firmly established for term and preterm neonates. Cord blood samples from 143 healthy newborns (78 full term and 65 premature) were analyzed with the Coulter counter. Platelet count and MPV were significantly greater (p < 0.05 and p < 0.001, respectively) in term versus preterm infants, while PDW was significantly less in term infants (p < 0.001). Platelet count and MPV correlated with gestational age, and platelet count also correlated with birth weight. There was a significant (p < 0.001) negative correlation of PDW with gestational age and birth weight. These data represent normal reference ranges for neonates and demonstrate significant variation with gestational age. (C) Lippincott-Raven Publishers.

Two normal appearing infants presented in the newborn period with elevated white blood cell counts and immature blast cells. Initial bone marrow karyotype analysis showed trisomy of chromosome 21 in all metaphases. In both patients blastemia spontaneously resolved and percentage of trisomy 21 cells decreased. One infant required multiple exchange transfusions and pericardotomy. The other patient had undifferentiated blasts and continued to have subtle hematopoietic abnormalities >2 years later. Both children have had normal growth and development. The clinical course of these patients emphasizes the need for aggressive supportive care without use of cytotoxic drugs in phenotypically normal new-borns with blastemia showing trisomy 21. (C) Lippincott-Raven Publishers.

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Advances in the treatment of patients with sickle cell disease have prolonged life span and increased the number of patients surviving into late adulthood. Many patients however, fail to reach their maximum potential because of the debilitating effects of recurrent pain. Our experience in a large pediatric-adult sickle cell program suggests that a comprehensive medical approach, utilizing adequate analgesic therapy and extensive counseling, can significantly reduce the morbidity and hospitalization due to chronic pain. We describe herein our approach to treatment of pain in sickle cell disease and report the results we have obtained by following it. (C) Lippincott-Raven Publishers.

Chemotherapy is an effective mode of therapy for children with histioeytosis, either alone or in conjunction with radiotherapy. This paper summarizes the results of clinical trials with both single-agent and multiple-agent treatment regimens and describes the prognostic variables which must be considered in designing future clinical trials. (C) Lippincott-Raven Publishers.

Sickle cell disease, a disease prevalent among the negroes all over the world, is discussed first from the historical point of view. Its geographical occurrence which is highest in the African subcontinent and lowest in the United States depends on the prevalence of malaria in the environment and intermarriage of races. The sociocultural background of Nigeria is discussed along with its existing health and educational problems. These are related to the profound clinical manifestation of sickle cell disease. Some pitfalls in the diagnosis and manifestation of sickle cell disease are mentioned. The problems to the use of certain drugs are highlighted. In solving some of the problems of sickle cell disease attention should be focused on continuous health education, establishing sickle cell clinics, and research aimed at improving the environment of patients in the underprivileged situations of the world where sickle cell disease poses problems. (C) Lippincott-Raven Publishers.

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Publication year: 2010
Source: Critical Reviews in Oncology/Hematology, In Press, Corrected Proof, Available online 12 March 2010

Patrick G., Morris , Monica N., Fornier

Recently, a greater understanding of tumor biology, including the use of genetic signatures, has led to a more precise classification of breast cancer. This has translated into a more granular assessment of risk-benefit calculations for the selection of patients for adjuvant therapies. For unselected patients, anthracyclines offer a survival benefit over non-anthracycline-based regimens, but are associated with long-term risks including congestive heart failure. Until recently, the benefit of anthracyclines in older patients with early breast cancer was largely extrapolated from studies involving younger cohorts. Emerging data now suggest that for appropriately selected older patients anthracyclines offer distinct benefits and are...

Publication year: 2010
Source: Critical Reviews in Oncology/Hematology, In Press, Corrected Proof, Available online 12 March 2010

Jean, Klastersky , Ahmad, Awada

The pertinent literature on clinical studies comparing the respective value of myeloid colony stimulating factors to pegfilgrastim as a prevention of febrile neutropenia in chemotherapy-treated cancer patients has been reviewed.Pegfilgrastim is definitely not inferior to filgrastim or other myeloid colony stimulating agents with respect to duration of grade IV neutropenia and delivery of full chemotherapy dose on time; several comparative studies indicate a trend to less frequent febrile neutropenia with pegfilgrastim.

Abstract Purpose  Hypogonadism has been linked with systemic inflammation and opioid use in males with advanced cancer. We aimed to investigate the interaction of gonadal status with systemic inflammation and opioids in determining nutritional status and prognosis in advanced pancreatic cancer. Methods  One hundred and seventy-five patients (92 males, 83 postmenopausal females) with unresectable pancreatic cancer were studied. Serum sex steroids (total testosterone [TT], calculated free testosterone [cFT], oestradiol, sex hormone binding globulin), gonadotropins (follicle-stimulating hormone and luteinising hormone) and pro-inflammatory mediators (C-reactive protein [CRP], interleukin-6 [IL-6], soluble tumour necrosis factor receptor 75 [sTNFR75]) were measured, and nutritional assessment and opioid use recorded. Results  Seventy-three percent of males were hypogonadal (by cFT definition). cFT correlated positively with BMI (r 2 = 0.349; p < 0.001) and grip strength (r 2 = 0.229; p = 0.034) and inversely with weight loss (r 2 = −0.287; p = 0.007), CRP (r 2 = −0.426; p < 0.001) and IL-6 (r 2 = −0.303; p = 0.004). CRP (p = 0.007), opioid dosage (p = 0.009) and BMI (p = 0.005) were independent determinants of cFT on ANOVA. Hypogonadal males demonstrated worsened survival compared with eugonadal patients (TT: OR of death = 2.87; p < 0.001; cFT: OR = 2.26; p = 0.011). Furthermore, male opioid use was associated with decreased TT (p < 0.001) and cFT (p < 0.001) and worsened survival (OR = 1.96; p = 0.012). In contrast, 18% of postmenopausal females exhibited premenopausal (“hyperoestrogenic”) oestradiol levels. Oestradiol correlated positively with sTNFR75 (r 2 = 0.299; p = 0.008). CRP (p < 0.001) was an independent determinant of oestradiol. Hyperoestrogenic females demonstrated worsened survival compared with eugonadal patients (OR = 2.43; p = 0.013). Conclusions  In males with pancreatic cancer, systemic inflammation and opioid use are associated with hypogonadism. Male hypogonadism and female hyperoestrogenism are associated with shortened survival in advanced pancreatic cancer.

• Content Type Journal Article
• Category Original Article
• DOI 10.1007/s00520-010-0832-y
• Authors
  • Richard J. E. Skipworth, University of Edinburgh, Royal Infirmary of Edinburgh Clinical and Surgical Sciences (Surgery) 51 Little France Crescent Edinburgh EH16 4SA UK
  • Alastair G. W. Moses, University of Edinburgh, Royal Infirmary of Edinburgh Clinical and Surgical Sciences (Surgery) 51 Little France Crescent Edinburgh EH16 4SA UK
  • Kathryn Sangster, University of Edinburgh, Royal Infirmary of Edinburgh Clinical and Surgical Sciences (Surgery) 51 Little France Crescent Edinburgh EH16 4SA UK
  • Catharine M. Sturgeon, Royal Infirmary of Edinburgh Department of Clinical Biochemistry 51 Little France Crescent Edinburgh EH16 4SA UK
  • Anne C. Voss, Abbott Laboratories Abbott Nutrition Research and Development 625 Cleveland Avenue Columbus OH 43215-1724 USA
  • Marie T. Fallon, Western General Hospital Department of Palliative Medicine, Edinburgh Cancer Centre Crewe Road Edinburgh EH4 2XR UK
  • Richard A. Anderson, University of Edinburgh Division of Reproductive and Developmental Science 47 Little France Crescent Edinburgh EH16 4TJ UK
  • James A. Ross, University of Edinburgh, Royal Infirmary of Edinburgh Clinical and Surgical Sciences (Surgery) 51 Little France Crescent Edinburgh EH16 4SA UK
  • Kenneth C. H. Fearon, University of Edinburgh, Royal Infirmary of Edinburgh Clinical and Surgical Sciences (Surgery) 51 Little France Crescent Edinburgh EH16 4SA UK

• Journal Supportive Care in Cancer
• Online ISSN 1433-7339
• Print ISSN 0941-4355

A 16-year-old female presented with symptoms consistent with constipation with no constitutional symptoms. Multiple different laxatives were attempted over 4 months and were unsuccessful. This thin female developed an impressively distended, nonacute abdomen within a 2-week period. Histology demonstrated a stage IV small cell carcinoma of the ovary. Her disease initially responded to treatment, but ultimately she relapsed and failed to respond to 2 other chemotherapy combinations, which were based on limited success found in the literature. She ultimately passed away 13 months after the diagnosis, demonstrating the poor prognosis and rapid spread of this rare disease. (C) 2010 Lippincott Williams & Wilkins, Inc.

Approximately 33% of adult patients with dermatomyositis develop malignancy with up to 42% presenting after the diagnosis has been made; careful evaluation for malignancy is often undertaken at the time of dermatomyositis diagnosis. This phenomenon has rarely been noted in pediatric patients and extensive workup for malignancy is not indicated in pediatric patients. In 1993, 6 cases were reported in which juvenile dermatomyositis/polymyositis (JDM/PM) appeared to be part of a paraneoplastic phenomenon. Our objective was to update the literature for reported cases of malignancy associated with JDM/PM; we reviewed the literature over the last 15 years and located 6 additional cases. In 9 of 12 reported patients an unusual physical finding such as splenomegaly or lymphadenopathy was noted at the time of diagnosis, and in the entire group, the malignancy occurred within a mean of 12 months. It is less likely that JDM/PM in pediatric patients is a paraneoplastic phenomenon as it is in adult patients. However, if the physical examination at the time of diagnosis of JDM/PM is atypical the presence of malignancy must be considered and a more in-depth evaluation to rule out malignancy should be performed prior to the initiation of treatment. (C) 2010 Lippincott Williams & Wilkins, Inc.

Although pancreatoblastoma (PB) is an extremely rare tumor, it is the most common pancreatic tumor in children. We reported 2 cases of PB treated at a medical center in Taiwan. One was a 3.5-year-old boy who presented with abdominal pain. Physical examination demonstrated abdominal masses. Multiple pancreatic and hepatic masses were noted by magnetic resonance imaging. He underwent surgical resection but tumor could not be removed completely, which was confirmed by the pathology. He received chemotherapy consisting of cisplatin and doxorubicin after the surgery but the tumor progressed rapidly. He died of progressive disease and sepsis. The other case was a 4-year-old boy presented with abdominal pain. Computed tomography showed pancreatic tumor. He underwent surgical resection and pathology showed PB. He received chemotherapy after complete tumor resection. He is disease free till now. Complete tumor resection is the major difference of these 2 patients and is the most important factor affecting the outcome. (C) 2010 Lippincott Williams & Wilkins, Inc.

No abstract available

Malignant perivascular epithelioid cell tumor (PEComa) was a kind of rare disease with malignant behavior and poor prognosis. Nearly all PEComas show immunoactivity for HMB45. The treatment strategy for malignant PEComa is still of controversy, especially in advanced or unresectable disease. We reported a 14-year-old patient of unresectable malignant PEComa, who was treated with multiple combined chemotherapies. The patient died of tumor 9 months after surgery, although 3 different combined chemotherapeutic regimens and antiangiogenic drug were used. Our case suggested that empirical chemotherapeutic regimens for soft tissue sarcomas might not be suitable for PEComa. (C) 2010 Lippincott Williams & Wilkins, Inc.

Erratum to: Glioblastoma cell growth is suppressed by disruption of fibroblast growth factor pathway signaling

• Content Type Journal Article
• Category Erratum
• DOI 10.1007/s11060-010-0155-3
• Authors
  • Watcharin Loilome, Johns Hopkins University Department of Neurosurgery, School of Medicine Baltimore MD 21231 USA
  • Avadhut D. Joshi, Johns Hopkins University Department of Neurosurgery, School of Medicine Baltimore MD 21231 USA
  • Colette M. J. ap Rhys, Johns Hopkins University Department of Neurosurgery, School of Medicine Baltimore MD 21231 USA
  • Sara Piccirillo, Università degli Studi Bicocca-Millan Milan 20126 Italy
  • Angelo L. Vescovi, Università degli Studi Bicocca-Millan Milan 20126 Italy
  • Gary L. Gallia, Johns Hopkins University Department of Neurosurgery, School of Medicine Baltimore MD 21231 USA
  • Gregory J. Riggins, Johns Hopkins University Department of Neurosurgery, School of Medicine Baltimore MD 21231 USA

• Journal Journal of Neuro-Oncology
• Online ISSN 1573-7373
• Print ISSN 0167-594X