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2.
Hum Pathol ; 43(7): 1028-36, 2012 Jul.
Article in English | MEDLINE | ID: mdl-22079355

ABSTRACT

Children with sickle cell anemia may undergo acute splenic sequestration. Splenectomy is performed in an attempt to reduce further events. Histologic studies of spleens have revealed the presence of granuloma-like nodules, known as Gamna-Gandy bodies with amorphous inclusions; however, their significance is unknown. The medical case records and histologic samples of consecutive children with sickle cell anemia treated with splenectomy between 2001 and 2007 at Our Lady's Children's Hospital, Dublin, were reviewed. Seventeen patients were identified. Gamna-Gandy bodies were studied by scanning electron microscopy and x-ray fluorescence spectroscopy. Gamna-Gandy bodies were identified in 7 (41%) patients, and amorphous inclusions were always seen. Patient age correlated significantly with Gamna-Gandy bodies (P = .002). Scanning electron microscopic analysis demonstrated the crystalline nature of Gamna-Gandy bodies and the chemical composition (C 47.1%; O(2) 29.7%; P 9.0%; K(+) 0.4%; Ca(2+) 6.4%; Fe(2+) 7.4%), whereas x-ray diffraction studied the structure (CaPO(4) ∙ FeOH). A crystal-formation gradient was observed, increasing from the red pulp to the white pulp. Our study shows that Gamna-Gandy bodies contain crystals and that their formation is age dependent. We also demonstrated the crystal structure and chemical composition and the relationship between Gamna-Gandy bodies and chest crises presplenectomy or postsplenectomy.


Subject(s)
Anemia, Sickle Cell/pathology , Spleen/pathology , Splenic Diseases/pathology , Adolescent , Anemia, Sickle Cell/surgery , Child , Child, Preschool , Female , Humans , Infant , Male , Spleen/surgery , Splenectomy , Splenic Diseases/surgery , Treatment Outcome
3.
Fetal Pediatr Pathol ; 30(3): 156-60, 2011.
Article in English | MEDLINE | ID: mdl-21355680

ABSTRACT

Congenital fibrosarcoma is a rare, soft tissue malignancy of infancy, most commonly involving the distal extremities. We report a case of congenital fibrosarcoma of the ileum in a 5-day-old boy who presented with an acute abdomen due to ileal perforation. Partial ileal resection was carried out with successful anastomosis. Grossly, the resected small bowel showed focal luminal stenosis with a thickened, indurated wall. Histology showed a transmural primitive spindle cell proliferation with a morphology consistent with congenital fibrosarcoma. The associated hallmark chromosomal translocation t(12;15)(q13;q25) was demonstrated by reverse transcriptase polymerase chain reaction.


Subject(s)
Fibrosarcoma/congenital , Ileal Neoplasms/congenital , Chromosomes, Human, Pair 12/genetics , Chromosomes, Human, Pair 15/genetics , Fibrosarcoma/genetics , Fibrosarcoma/pathology , Fibrosarcoma/surgery , Humans , Ileal Neoplasms/genetics , Ileal Neoplasms/pathology , Ileal Neoplasms/surgery , Infant, Newborn , Intestinal Perforation/etiology , Intestinal Perforation/surgery , Male , Oncogene Fusion , Oncogene Proteins, Fusion/genetics , Translocation, Genetic
4.
Biopreserv Biobank ; 9(4): 389-398, 2011 Dec.
Article in English | MEDLINE | ID: mdl-23386926

ABSTRACT

The Saint James's Hospital Biobank was established in 2008, to develop a high-quality breast tissue BioResource, as a part of the breast cancer clinical care pathway. The aims of this work were: (1) to ascertain the quality of RNA, DNA, and protein in biobanked carcinomas and normal breast tissues, (2) to assess the efficacy of AllPrep(®) (Qiagen) in isolating RNA, DNA, and protein simultaneously, (3) to compare AllPrep with RNEasy(®) and QIAamp(®) (both Qiagen), and (4) to examine the effectiveness of Allprotect(®) (Qiagen), a new tissue stabilization medium in preserving DNA, RNA, and proteins. One hundred eleven frozen samples of carcinoma and normal breast tissue were analyzed. Tumor and normal tissue morphology were confirmed by frozen sections. Tissue type, tissue treatment (Allprotect vs. no Allprotect), extraction kit, and nucleic acid quantification were analyzed by utilizing a 4 factorial design (SPSS PASW 18 Statistics Software(®)). QIAamp (DNA isolation), AllPrep (DNA, RNA, and Protein isolation), and RNeasy (RNA isolation) kits were assessed and compared. Mean DNA yield and A(260/280) values using QIAamp were 33.2 ng/µL and 1.86, respectively, and using AllPrep were 23.2 ng/µL and 1.94. Mean RNA yield and RNA Integrity Number (RIN) values with RNeasy were 73.4 ng/µL and 8.16, respectively, and with AllPrep were 74.8 ng/µL and 7.92. Allprotect-treated tissues produced higher RIN values of borderline significance (P=0.055). No discernible loss of RNA stability was detected after 6 h incubation of stabilized or nonstabilized tissues at room temperature or 4°C or in 9 freeze-thaw cycles. Allprotect requires further detailed evaluation, but we consider AllPrep to be an excellent option for the simultaneous extraction of RNA, DNA, and protein from tumor and normal breast tissues. The essential presampling procedures that maintain the diagnostic integrity of pathology specimens do not appear to compromise the quality of molecular isolates.

5.
Int J Gynecol Pathol ; 27(1): 74-8, 2008 Jan.
Article in English | MEDLINE | ID: mdl-18156979

ABSTRACT

The Mirena intrauterine system (IUS) has improved the options available to women with menorrhagia. However, in some women, IUS treatment fails to reduce menstrual flow, and surgical treatment, including hysterectomy, is necessary. We have reviewed the histopathological findings on the uteri of 44 women undergoing hysterectomy because of menorrhagia after unsuccessful IUS treatment to assess whether a potentially unresponsive cohort could be identified. A retrospective review of 44 hysterectomy specimens was performed between October 1999 and April 2006 on women who underwent unsuccessful treatment of menorrhagia with the IUS. The patients' ages ranged from 30 to 53 years (median age, 43 years; all were premenopausal). Most women (60%) had the expected appearance of atrophy of the endometrial glands and pseudodecidual stromal reaction. Thirty hysterectomy specimens contained benign leiomyomata with associated reduced reactivity in the uterine cavity and incomplete suppression of the endometrium. In some cases (n = 10), the fibroids had displaced the IUS in the uterine cavity. Fourteen specimens showed adenomyosis, of which 8 also contained fibroids. In addition to leiomyomas, 1 specimen had an atypical polypoid adenomyoma and 1 had a benign adenomatoid tumor. Two specimens had endometrial hyperplasia for which the IUS was unsuccessful in controlling bleeding. Two specimens showed intrauterine misplacement of the IUS. Only 6 women (13.6%) had no histological abnormalities. Most women (86%) undergoing hysterectomy because of abnormal uterine bleeding with a Mirena IUS in situ had uterine abnormalities, as revealed by pathological review. Although recent reports have indicated that the IUS can be used successfully in the treatment of menorrhagia due to uterine fibroids, most cases of hysterectomies in this series after failed IUS suppression of menorrhagia contained uterine fibroids.


Subject(s)
Intrauterine Devices, Medicated , Levonorgestrel/therapeutic use , Menorrhagia/drug therapy , Uterus/drug effects , Uterus/pathology , Endometrial Hyperplasia/complications , Endometrial Hyperplasia/pathology , Endometriosis/complications , Endometriosis/pathology , Female , Humans , Hysterectomy , Leiomyoma/complications , Leiomyoma/pathology , Menorrhagia/etiology , Menorrhagia/surgery , Retrospective Studies , Treatment Failure , Uterine Neoplasms/complications , Uterine Neoplasms/pathology
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