Renal transplant in a patient of severe hemophilia.

Hemophilia is a rare disorder that is difficult to diagnose and manage. The prevalence of end-stage renal disease is increasing in hemophilic patients because of improved life expectancy. Renal transplant surgery in such patients is often complicated by adverse hematological events such as bleeding and intravesical thrombosis, both with a risk of renal allograft rejection. We report a case of a 46-year-old hemophilia A patient on hemodialysis who underwent renal transplant and show that although challenging, renal transplant in hemophilic patients is possible.

Multicenter, randomized phase II trial of bevacizumab plus folinic acid, fluorouracil, gemcitabine (FFG) versus bevacizumab plus folinic acid, fluorouracil, oxaliplatin (FOLFOX4) as first-line therapy for patients with advanced colorectal cancer.

PURPOSE: To assess safety and efficacy of folinic acid, 5-fluorouracil, gemcitabine (FFG) and folinic acid, fluorouracil, oxaliplatin (FOLFOX4) regimens with added bevacizumab as first-line treatment in patients with advanced colorectal cancer (CRC). PATIENTS AND METHODS: Patients with Stage III unresectable or Stage IV adenocarcinoma of the colon or rectum were randomly assigned to either FFG weekly for 6 weeks of an 8-week cycle or FOLFOX4 every 2 weeks. After FDA approval, bevacizumab 5 mg/kg was added every 2 weeks. Treatment continued until disease progression. Planned enrollment was 190 patients. Primary endpoint was overall response rate (ORR); secondary endpoints included evaluation of adverse events, time to progression (TTP), and overall survival (OS). Disease Control Rate (DCR; % of patients with complete or partial responses or stable disease) was a post hoc analysis. RESULTS: The trial was stopped prematurely due to low enrollment. Of 84 enrolled patients (42 to each arm), 36 patients (18 in each arm) received bevacizumab. ORR was greater (P = .002) for FOLFOX4 (17/42; 40.5%) than for FFG (4/42; 9.5%); however, TTP, OS, and DCR results were not statistically different comparing FOLFOX4 and FFG. Peripheral neuropathy was more frequent (P = <.001) with FOLFOX4 (18/42; 42.9%) than with FFG (1/42; 2.4%). CONCLUSIONS: FFG and FOLFOX4 were generally well tolerated. Based on ORR, FOLFOX4 was superior to FFG. However, differences in TTP and OS comparing regimens were inconclusive. General use of gemcitabine as a biomodulator of 5-fluorouracil in CRC cannot be recommended at this time and the regimen remains investigational.

A prospective longitudinal study to evaluate bone health, implication of FRAX tool and impact on quality of life (FACT-P) in advanced prostate cancer patients.

BACKGROUND: Androgen-deprivation therapy (ADT) as a treatment modality in advanced prostate cancer has deleterious effect on bone mineral density (BMD) and quality of life (QOL). Using FRAX (Fracture Risk Assessment) model, candidates at high risk of fractures can be predicted and appropriate treatment can be initiated at early step to prevent skeletal-related events. Objectives of the present study were to evaluate bone health, implication of FRAX tool in advanced prostate cancer and to see the impact of ADT and Bone-directed therapy (BDT) on FRAX and FACT-P QOL scores. MATERIAL & METHOD: We conducted a prospective longitudinal study of 83 localized and metastatic prostate cancer patients from March 2017 to Dec 2020. FRAX tool using BMD femoral neck (GE-Lunar) was used to compute the probability of 10-year Major osteoporotic fracture (MOF) and hip fracture risk %. Patients who received monthly Zolendronic acid with or without Vitamin-D/calcium supplementation were classified as BDT group. FRAX and FACT-P were measured at baseline and 12 months follow-up and compared between different therapeutic modalities to see the impact on clinical outcomes. RESULTS: Majority of patients had skeletal metastasis (78.3%) and high-grade disease at presentation. Secondary osteoporosis was the most commonly (82.05%) observed clinical risk factor (CRF) followed by smoking (19.23%). Hip fracture risk ≥3% accounted for larger proportion of patients than did MOF risk ≥20% (21.2% and 2.5%, respectively). Statistically significant reduction was observed in both MOF and hip fracture risk in BDT group, while worsening on ADT. ADT duration correlated positively with both MOF and hip fracture risk (R2=0.148, P<0.001 and R2=0.164, P<0.001, respectively). FRAX score accurately predict future fracture events in majority (80%) of high-risk patients. Statistically and clinically significant worsening in PWB, EWB, PCS, FACT-P Total, FACT-P TOI and FAPSI scores were observed in patients on ADT. Statistically and clinically significant improvement was noted in physical well-being in BDT group. However, other QOL domains and FACT-P total scores remained stable. CONCLUSIONS: ADT caused duration depended worsening of FRAX and FACT-P score in these patients while improvements of FRAX were seen on BDT. FRAX tool is advantageous in identifying the patients who require early intervention or therapy to decrease skeletal-related events.

Abdominal aortitis after use of granulocyte colony-stimulating factor.

Granulocyte colony-stimulating factor (G-CSF) is a recombinant human glycoprotein that promotes proliferation and differentiation of granulocytic-committed progenitors. It is commonly used to treat neutropenia and is generally well tolerated. Occurrences of rare but serious adverse events in association with the use of G-CSF have been described. We report the case of a 54-year-old male with squamous cell carcinoma of the lung who developed abdominal aortitis following the use of G-CSF. Other possible aetiological conditions were excluded based on laboratory and radiological evaluations. To our knowledge, this represents the second case report demonstrating an association between aortitis and the use of G-CSF.

Recurrent Metastatic Eccrine Porocarcinoma: A Case Report and Review of the Literature.

BACKGROUND Eccrine porocarcinoma, or malignant eccrine poroma, is a rare primary skin tumor that develops in the sixth and seventh decades of life, and can present as a painless and solitary nodule. Histopathology is required to confirm the diagnosis. A rare case is presented of metastatic eccrine porocarcinoma, occurring four years after surgical excision of the primary scalp tumor, and includes a review of the literature. CASE REPORT A 67-year-old man initially presented with a scalp lesion that was non-painful, exophytic, and pigmented. Following complete excision, histopathology confirmed the diagnosis of eccrine porocarcinoma with clear resection margins. Four years later, he presented with discrete erythematous patches and plaques, in a zosteriform distribution, in the skin of the right neck, shoulder, and chest. A biopsy and histopathology of the skin rash confirmed metastatic eccrine porocarcinoma. A positron-emission tomography-computed tomography (PET-CT) scan identified areas of hypermetabolic activity, with a standardized uptake value (SUV) of 12, and an infiltrating soft tissue tumor in the right suboccipital region. Surgical resection of the suboccipital mass, followed by histopathology, confirmed metastatic eccrine porocarcinoma. During a postoperative ear, nose, and throat (ENT) examination, he was found to have metastases in the right ear canal. The patient received five cycles of chemotherapy, but later developed renal failure and eventually chose palliative care. CONCLUSIONS A rash-like presentation of skin metastasis to the trunk and metastasis to the ear from a primary eccrine porocarcinoma is rare. Early diagnosis and adequate surgical resection are recommended to reduce patient mortality.

Recurrent intrathoracic dedifferentiated liposarcoma: A case report and literature review.

BACKGROUND: Liposarcoma (LPS) is the second most common type of soft tissue sarcoma, accounting for approximately 15-20% of all the sarcomas. Primary intrathoracic LPS, however, is quite rare. LPS is a malignant mesenchymal tumor, comprised of lipogenic tissue with varying degrees of atypia. It can be subclassified into well-differentiated LPS (WDLPS), myxoid LPS (MLPS)/round cell LPS, pleomorphic LPS (PLPs), and dedifferentiated LPS (DDLPS), based on the histology. CASE PRESENTATION: A 76-year-old male patient initially presented to the emergency room with a complaint of precordial chest pain for one month. Computed tomography (CT) of his chest showed a large, 8 cm × 8 cm x 10 cm, supradiaphragmatic, complex solid mass in the lower left hemithorax, along the anterior chest wall. Chest wall mass excision revealed dedifferentiated LPS, with excision of margins. Positron emission tomography (PET) scan did not show metastatic disease. Seven months later, he presented with shortness of breath, and CT of the chest showed large, left pleural-based masses, causing compression of surrounding structures. He was not a candidate for surgical resection. This patient subsequently failed chemotherapy and opted for hospice. CONCLUSION: Intrathoracic LPS is a rare tumor. Recurrence is higher with dedifferentiated histology forms. Radical surgery with excision of margins is the primary recommended treatment.

Diclofenac-induced thrombotic thrombocytopenic purpura with concomitant complement dysregulation: a case report and review of the literature.

BACKGROUND: Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are two forms of thrombotic microangiopathies. They are characterized by severe thrombocytopenia, microangiopathic hemolysis, and thrombosis, leading to a systemic inflammatory response and organ failure. Plasmapheresis is used to treat thrombotic microangiopathies. A different entity known as atypical hemolytic uremic syndrome has garnered more clinical recognition because reported cases have described that it does not respond to standard plasmapheresis. Diclofenac potassium is a non-steroidal anti-inflammatory drug that is used to treat pain. CASE REPORT: A 35-year-old Hispanic man presented to our emergency department with complaints of generalized malaise, fever, and an evanescent skin rash. During admission, he reported the use of diclofenac potassium for back pain on a daily basis for 1 week. He was noted to have peripheral eosinophilia, so he was admitted for suspected drug reaction involving eosinophilia and systemic symptoms. His initial laboratory work-up showed microangiopathic hemolytic anemia and thrombocytopenia. He also experienced a seizure, encephalopathy, and had a PLASMIC score of 7, thus raising concerns for thrombotic thrombocytopenic purpura. He underwent emergent plasmapheresis, which improved his clinical condition. The diagnosis was confirmed by assessing the levels of disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13, which was less than 3%. In addition, his skin biopsy was positive for patchy complement deposition, demonstrating complement dysregulation. CONCLUSION: Thrombotic thrombocytopenic purpura is a rare condition that can be acquired. Our case is rare because it represents the first report of diclofenac potassium-induced thrombotic thrombocytopenic purpura with subjacent complement activation and dysregulation. Early recognition and aggressive management led to a favorable outcome.

Lung Cancer Presenting as Skin Metastasis of the Back and Hand: A Case Series and Literature Review.

Lung cancer has the highest mortality of all cancers in the United States. The incidence of lung cancer with metastases to the skin varies between 1-12%, with the highest incidence seen in men. Here, we present two cases of lung cancer presenting as skin metastasis. The first patient was an 80-year-old African American male who presented to the hospital for evaluation of a right upper back mass. A few months prior to admission, he was found to have a left lung mass on CT scan of the chest, he underwent biopsy which showed poorly differentiated SCC of the lung. He also had a skin biopsy which showed poorly differentiated carcinoma in the dermis consistent with metastatic SCC. He was started on chemotherapy, but could not tolerate it. He was accepted to hospice. The second patient was a 78-year-old Hispanic female who presented to the hospital with dyspnea, and a dry cough. Upon physical examination, a 2 × 2 cm ulcerated, wart-like nodule on the right palm was noted. Subsequent CT scan of the chest showed a partial collapse of the right middle lobe. A biopsy of the hand mass revealed well-to-moderately differentiated metastatic SCC favoring lung origin. A bronchoscopy biopsy showed invasive SCC. Subsequently her condition worsened and she passed away. Metastasis to the skin is an unusual presenting symptom of lung cancer. It is therefore essential to consider metastasis as a diagnosis in a patient with both a skin lesion and a smoking history.

Co-Occurrence of Myeloid Sarcoma of the Lymph Node and Acute Monocytic Myeloid Leukemia: A Case Report and Literature Review.

BACKGROUND: Acute myeloid leukemia (AML) is the most common leukemia in adults. According to the French-American-British (FAB) system, monocytic leukemia is classified as M5. Myeloid sarcoma further occurs in 3-5% of AML. This is defined as an extramedullary tumor of myeloid cells in the lymph nodes, soft tissues, periosteum, bone, central nervous system (CNS), spinal cord, intestine, mediastinum, prostate, uterus, or ovaries. CASE PRESENTATION: Here, we describe the case of a 29-year-old female who presented with fever, swelling of gums, neck pain, and weakness, which had persisted for 1 week. The patient had a white blood cell (WBC) count of 53.5 K/µL, and a peripheral smear revealed a myeloid blast cell (blast) percentage of 8%. Computed tomography (CT) of the neck indicated lymphadenopathy. Fine needle aspiration of the cervical lymph node showed groups of atypical immature myeloid cells, mixed with occasional megakaryocytes, and infiltration of eosinophilic myeloid cells into the lymph node, consistent with myeloid sarcoma. Flow cytometry analysis revealed intermediate to large circulating blasts, with irregular nuclei, fine chromatin, and distinct nucleoli, indicative of AML, with monocytic differentiation. The patient responded well to chemotherapy with fludarabine, cytarabine, granulocyte colony stimulating factor (G-CSF), and idarubicin; WBC counts returned to normal and patient was discharged to home. CONCLUSION: Myeloid sarcoma of the lymph node is a rare co-occurrence with AML. Results of our study are consistent with the conclusion that early diagnosis and appropriate treatment improve survival.

A Rare Case of Sarcomatoid Carcinoma of the Lung with Spine Metastasis, Including a Literature Review.

BACKGROUND Sarcomatoid carcinoma is a rare, aggressive, malignant cancer composed of sarcoma and sarcoma-like components, and can occur in different organs such as the thyroid gland, bone, skin, breast, pancreas, liver, urinary tract, and lung. Pulmonary sarcomatoid carcinoma accounts for only a small percentage of lung cancers and has histological variants that include pleomorphic carcinoma, giant cell carcinoma, spindle cell carcinoma, carcinosarcoma, and pulmonary blastoma. CASE REPORT Here, we present a case of sarcomatoid carcinoma in a 63-year-old HIV-positive Hispanic male who presented with back pain, dry cough, and weight loss. A CT scan of his chest showed an ovoid mass in the lower lobe of the left lung, and an MRI of the spine showed a left lateral paraspinal soft tissue mass causing central canal stenosis and mild cord compression. The patient underwent laminectomy and resection of the spinal mass. A transthoracic needle biopsy of the lung and spinal masses had similar histopathology, and were indicative of sarcomatoid carcinoma. CONCLUSIONS We report a rare case of sarcomatoid carcinoma involving both the lung and spinal cord in the same patient. Sarcomatoid carcinomas of the lung have poor prognosis and are aggressive cancers. Moreover, our case also had the co-occurrence of HIV and sarcomatoid carcinoma.

Thiazolidin-4-one Derivatives as Central Nervous System Potential Agents.

A heterocyclic compound is one which possesses a cyclic structure with at least two different kinds of hetero atoms in the ring. Nitrogen, oxygen, and sulphur are the most common heteroatoms. derivatives are an important class of heterocyclic compounds and play a vital role due to their wide range of biological activities and industrial importance. 4-Thiazolidinones are always being an attraction point for researchers because of its efficiency towards various pharmacological usages. This review is an endeavor to highlight the progress in the central nervous system activity of the 4-thiazolidinone derivatives.

Recurrent cerebral embolism associated with indwelling catheter in the presence of anomalous neck venous structures.

Persistent left superior vena cava (PLSVC) is the most common central venous anomaly in the thorax, which may remain asymptomatic throughout life. Indwelling catheters that are increasingly used in oncology practice may result in complications in the presence of PLSVC. The authors report an unusual case of recurrent ischemic attacks each time a portacath was accessed in a patient with breast cancer and PLSVC. This case highlights the importance of suspecting this condition in the presence of clinical and radiographic clues to prevent complications.