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Efficiency and effectiveness of using nonmedical staff during an urgent mass prophylaxis response.

Using a simulated anthrax scenario, the Philadelphia Department of Public Health tested the readiness of a nonmedical closed point-of-dispensing (POD) site to see how rapidly and accurately it could provide medication to its internal population. This closed POD had developed and exercised its mass prophylaxis plan in conjunction with the local health department twice before, and the department was interested in assessing the impact of having no onsite department involvement. Two sessions were conducted as part of the overall exercise. In session 1, agency staff ran POD operations with no depar…

Modeling Legionnaires' disease outbreaks: estimating the timing of an aerosolized release using symptom-onset dates.

BACKGROUND: Over the last 30 years, there have been a number of reported Legionnaires' disease outbreaks resulting from the release of causative organisms from aerosol-producing devices. METHODS: We model a Legionnaires' disease epidemic curve as the convolution of an infection-time distribution (representing the aerosolized release) and an incubation-period distribution. The model is fitted to symptom-onset data from specific outbreaks to estimate the start and end dates of the release. We also develop this retrospective "back-calculation" model into a prospective "real-time" model that can

Abnormal chromatin clumping in leucocytes of Ph positive chronic myeloid leukemia cases - extending the morphological spectrum.

The syndrome of abnormal chromatin clumping is largely a morphological entity characterized by exaggerated chromatin clumping seen in the neutrophils. According to the recent World Health Organization (WHO) classification, it is categorized as a variant of atypical chronic myeloid leukemia (aCML) or Ph-negative CML. Most of the cases reported in literature have been negative for the Ph chromosome or the BCR-ABL gene. Till date, Ph positivity has been demonstrated in just one case. We report two more Ph-positive CML cases with abnormal chromatin clumping in neutrophils. To the best of our knowl

Случай острой лимфобластической лейкемии, развивающейся после диагностирования миелодипластического синдрома после леналидомидной терапии в случае множественной миеломы.

Острая лимфоболическая лейкемия (ALL) после лечения линалидомида (LEN) встречается редко. 64-летний мужчина развил миэлодизпластический синдром с избыточными взрывами-2 (MDS-EB-2) через шесть лет после терапии LEN после аутологической трансплантации стволовых клеток в случае множественной миеломы, а затем через 14 месяцев Филадельфия хромосома-отрицательная В-клетовая острая лимфоболическая лейкемия (АЛЛ). Геномический анализ диагноза выявил PRPF8, XBP1 и BCORL1 мутации с исключениями IKZF1 и PAX5. В архивных образцах с миэломы или фаз MDS не было обнаружено никаких аномалий. Он достиг полной

Совпадение в аннотации оригинала
Acute lymphoblastic leukemia (ALL) following lenalidomide (LEN) therapy is rare. A 64-year-old man developed myelodysplastic syndrome with excess blasts-2 (MDS-EB-2) six years after LEN maintenance therapy following autologous stem cell transplantation for multiple myeloma, followed by Philadelphia chromosome-negative B-cell acute lymphoblastic leukemia (ALL…

Novel e4a2 BCR∷ABL1 transcript with insertion of CSE1L exons 9 and 10 in a CML patient: a case report.

The BCR::ABL1 fusion gene, resulting from the Philadelphia (Ph) chromosome, is the defining feature of Chronic Myeloid Leukemia (CML). The fusion transcript typically results from the juxtaposition of ABL1 exons 2 or 3 and BCR exons 1, 13, 14 or 19, while exons 6 and 8 are less frequently involved. Here, we report the first case of a translocation in a patient with newly diagnosed chronic-phase CML harboring a novel e4a2 BCR::ABL1 fusion gene. This unique fusion includes a 298 bp insertion, derived from a CSE1L gene exons 9 and 10, at the fusion site. The patient showed resistance to first-lin…

Chronic myeloid leukemia with atypical transcript e8a2: a case report and literature review.

Chronic myeloid leukemia (CML) is characterized by the BCR::ABL1 fusion gene, resulting from Philadelphia (Ph) chromosome rearrangements that generate abnormal t (9; 22)(q34;q11) translocations. The most common fusion variants are e13a2 and e14a2. We diagnosed a case of CML with the rare e8a2 fusion variant using real-time quantitative PCR and sequencing. Although the e8a2 variant is increasingly reported in CML, comprehensive retrospective analyses remain scarce. Through a systematic review of published e8a2 BCR::ABL1 cases, we summarized the classification, treatment outcomes, and prognostic…

Anterior Atlas Fracture After C1 Laminectomy Which Was Successfully Treated by Brace Therapy: A Case Report.

CASE: We report a case of a 67-year-old male patient who underwent a C1 laminectomy with laminoplasty at C2-3 because of cervical ossification of the posterior longitudinal ligament. At 6 weeks after surgery, neck pain occurred after minor trauma without neurological deterioration. Computed tomography (CT) demonstrated C1 anterior atlas fracture with a 3-mm gap. After a 3-month brace therapy using a Philadelphia collar, the patient's neck pain disappeared with complete bone union according to the CT. CONCLUSION: Among 14 cases that were reported previously, none of the adult patients achieved…

Periodontoid pseudotumoral lesions.

BACKGROUND: Periodontoid pseudotumoral lesions (PPL) are an uncommon cause of cervical pain and myelopathy. In addition, they may be associated with atlantoaxial instability (AAI). CASE DESCRIPTION: Two patients over 60 years of age presented with neck pain alone. Their MR scans showed expansive lesions involving the odontoid process. One patient with AAI required an occipitocervical arthrodesis, while the other patient without instability was managed with an external orthosis (Philadelphia collar). Both of them experienced full resolution of pain and remained neurologically intact an average…

Multiple Reactivations of Viral Infections Followed by Cerebral Toxoplasmosis After Allogeneic Hematopoietic Stem Cell Transplantation in an Adolescent With Ph(+) Acute Lymphoblastic Leukemia: A Case Report.

After allogeneic hematopoietic stem cell transplantation (allo-HSCT), especially from an unrelated donor, infectious complications are frequ … f multiple viral infections or reactivations and cerebral toxoplasmosis in a 17-year-old youth with Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL) treated with allo-HSCT who suffered multiple viral infections followed by cerebral toxoplasmosis.

Unilateral optic disk edema with central retinal artery and vein occlusions as the presenting signs of relapse in acute lymphoblastic leukemia.

CLINICAL CASE: A 39-year-old man with Philadelphia chromosome-positive acute lymphoblastic leukemia (LAL Ph+) developed progressive vision loss to no light perception in his right eye. He had optic disk edema and later developed central artery and vein occlusions. Pan-photocoagulation, as well as radiotherapy of the whole brain were performed in several fractions. Unfortunately the patient died of hematological relapse 4 months later. DISCUSSION: Optic nerve infiltration may appear as an isolated sign of a leukemia relapse, even before a hematological relapse occurs. Leukemic optic neuropathy…

Myeloproliferative neoplasia: a review of clinical criteria and treatment.

Essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF) belong to the group of Philadelphia chromosome-negative myeloproliferative neoplasia (Ph- MPN). MPNs are clonal bone marrow stem cell disorders characterised by a proliferation of one or more of the myeloid, erythroid or megakaryocytic cell lines. Due to the different affected cell lines, MPNs show typical clinical and histological features. In 2005, a mutation in the JAK2 gene was discovered which generated more insight into the pathogenetic working mechanism of MPNs. However, the treatment of MPN patients …

Perinatal testicular torsion: preoperative radiological findings and the argument for urgent surgical exploration.

Perinatal testicular torsion is an infrequent event, the management of which has been controversial. Occurrence is rare, estimated at 1 in 7500 newborns (Kaplan, G. W., Silber, I.: Neonatal torsion--to pex or not? In: Urologic surgery in neonates and young infants. Edited by King, L.R. Philadelphia: W.B. Saunders Co., 1988; Chapter 20, pp. 386-395). The frequency of bilateral perinatal torsion is up to 22% (J Urol. 2005;174:1579). Here, we describe two cases of bilateral asynchronous perinatal torsion, in which the only presenting abnormality on exam after birth was a unilateral scrotal mass. …

[Molecular genetic typing of Legionella pneumophila serogroup 1 isolated in town Verkhnyaya Pyshma using international standards].

Strains of Legionella pneumophila Pyshma-1 and Pyshma-2 were typed according to the international standard - STB protocol developed by EGWLI. Allelic profile of the strains was determined. Identity of strains on the locus pilE, which codes protein important for virulence of bacterium, was shown. Close similarity of nucleotide sequences in Pyshma-1 and Pyshma-2 strains (mainly, 98-100%) was noted. Both strains differed more from reference strain Philadelphia-1 ATCC 33152. Maximal differences (5-6%) were observed in fragment of mompS gene. The study revealed considerable need for conducting of s…

A gene expressed exclusively in acute B lymphoblastic leukemias.

Representational difference analysis, a cDNA subtraction approach, was used to identify genes that are expressed in acute leukemia but not in normal hematological tissues. We isolated a cDNA fragment from a cell line derived from a B cell acute lymphoblastic leukemia bearing two Philadelphia chromosomes. The cDNA derives from an orphan gene that was named BLACE. BLACE is located in region 7q36 and encodes a major 5.3-kb transcript and several alternatively spliced minor transcripts. Significant expression of BLACE was detected by RT-PCR and quantitative RT-PCR in bone marrow samples from B cel…

Three-wheeled vehicle injuries in children.

Injuries associated with nonmotorized three-wheeled vehicles were prospectively evaluated through a standard questionnaire during the summer months at the emergency department of the Children's Hospital of Philadelphia, Pa. Forty-four children, including 32 boys, with a mean age of 3.77 years, reported such injuries. Most fell (36.4%), but a second vehicle was often involved. Most children (33 of 44) were on low-slung Big Wheels. Head, neck, and dental injuries accounted for the majority. Most injuries were not serious. Two patients were admitted; both had fractures, one of them had a concussi…

Bosutinib: Pediatric First Approval.

Bosutinib (BOSULIF®), an orally administered BCR-ABL tyrosine kinase inhibitor (TKI) developed by Pfizer Inc., is well established in the EU and the USA as a treatment for adults with newly diagnosed (ND) chronic phase (CP) Philadelphia chromosome-positive (Ph+) chronic myelogenous leukemia (CML), and for CP, accelerated phase and blast phase Ph+ CML that is resistant or intolerant (R/I) to prior therapy. In September 2023, based on clinical data from patients aged ≥ 1 to < 18 years, bosutinib was approved in the USA for the treatment of pediatric patients aged ≥ 1 year with CP Ph+ CML that is…

Erythema annulare centrifugum in a case of chronic myeloid leukemia.

Chronic myeloid leukemia (CML), BCR-ABL1-positive, is classified as a myeloproliferative neoplasm predominantly composed of proliferating granulocytes and determined to have the Philadelphia chromosome/translocation t (9;22)(q34;q11.2). Skin is said to be the mirror to systemic diseases, with skin manifestations ranging from nonspecific cutaneous lesions to specific lesions. The cutaneous manifestations of CML are generally reddish-brown to violaceous papules and nodules, indurated or hemorrhagic plaques, perifollicular acneiform papules, bullae, and palpable purpura. Other unusual manifestati…

Unnatural Resources: The Colonial Logic of the Holmesburg Prison Experiments.

This article focuses on medical trials performed by Dr. Albert Kligman on the inmates of Philadelphia's Holmesburg Prison between 1951 and 1974, which have been widely criticized as exploitative. I seek to investigate the mechanics behind the "ethical blind spot" that enabled the American medical community to laud Kligman for his efforts while simultaneously condemning the medical atrocities of the Holocaust and supporting the development of the Nuremberg Code. I argue that this nonrecognition hinges on a colonial logic by which certain populations are produced as waste, both rhetorically and …

Chronic Myeloid Leukemia, Version 1.2019, NCCN Clinical Practice Guidelines in Oncology.

Chronic myeloid leukemia (CML) is defined by the presence of Philadelphia chromosome (Ph), resulting from a reciprocal translocation between chromosomes 9 and 22 [t(9;22] that gives rise to a BCR-ABL1 fusion gene. CML occurs in 3 different phases (chronic, accelerated, and blast phase) and is usually diagnosed in the chronic phase. Tyrosine kinase inhibitor (TKI) therapy is a highly effective first-line treatment option for all patients with newly diagnosed chronic phase CML (CP-CML). The selection TKI therapy should be based on the risk score, toxicity profile of TKI, patient's age, ability t…