Case Series and Review of Hematological and Non-Hematological Malignancies in Aging Patients with Sickle Cell Disease in the Hydroxyurea Era.

Survival of adult patients with sickle cell disease has increased progressively since the 1970s. Aging patients with sickle cell disease are at risk of developing comorbidities that are not due to sickle cell disease itself, including malignancies. Many studies tried to assess the incidence of malignancy in patients with sickle cell disease. However, no studies have been done to evaluate cancer incidences in aging sickle cell patients, especially in the hydroxyurea (HU) era. In this review, we assessed the prevalence of malignancies in aging patients with sickle cell disease at our institution with or without HU therapy. Retrospective analysis of hospital records identified patients who had been diagnosed to carry sickle cell disease and malignancies before 2020 using the International Statistical Classification of Diseases and Related Health Problems (ICD-10) coding. Four hundred and eighty-three sickle cell disease patients were seen in our inpatients/outpatients offices. Among these, 12 sickle cell disease patients had a confirmed diagnosis of malignancy. The patients were classified into three categories based on age groups: four patients who were 60 years and older had multiple myeloma. Solid tumors were found in 5/6 patients, aged 40-60 who had the Hb S (HBB: c.20A>T) (ßS/ßS) genotype with signs of iron overload. Two patients, aged 25 and 35, had hematological malignancies. The number of patients on HU was too small to make any comment on relationship to malignancy or mortality. This study is only one institution's experience, further investigation on a larger scale is needed to look into cancer incidences in this population.

Hepatitis C risk assessment, testing and referral for treatment in urban primary care: role of race and ethnicity.

AIM: To determine rates of hepatitis C (HCV) risk factor ascertainment, testing, and referral in urban primary care practices, with particular attention to the effect of race and ethnicity. METHODS: Retrospective chart review from four primary care sites in Philadelphia; two academic primary care practices and two community clinics was performed. Demographics, HCV risk factors, and other risk exposure information were collected. RESULTS: Four thousand four hundred and seven charts were reviewed. Providers documented histories of injection drug use (IDU) and transfusion for less than 20% and 5% of patients, respectively. Only 55% of patients who admitted IDU were tested for HCV. Overall, minorities were more likely to have information regarding a risk factor documented than their white counterparts (79% vs 68%, P < 0.0001). Hispanics were less likely to have a risk factor history documented, compared to blacks and whites (P < 0.0001). Overall, minorities were less likely to be tested for HCV than whites in the presence of a known risk factor (23% vs 35%, P = 0.004). Among patients without documentation of risk factors, blacks and Hispanics were more likely to be tested than whites (20% and 24%, vs 13%, P < 0.005, respectively). CONCLUSION: (1) Documentation of an HCV risk factor history in urban primary care is uncommon, (2) Racial differences exist with respect to HCV risk factor ascertainment and testing, (3) Minority patients, positive for HCV, are less likely to be referred for subspecialty care and treatment. Overall, minorities are less likely to be tested for HCV than whites in the presence of a known risk factor.

Prevalence of HCV risk factors in Hispanic-American sub populations.

To assess the prevalence of HCV risk factors among Hispanic-American subpopulations in Philadelphia. Patients from four primary care practices in Philadelphia were enrolled. Demographics and HCV risk factors were ascertained using a self-administered questionnaire. Five hundred and three patients who identified themselves as Hispanic or Latino were included in the study. Approximately half were born in Puerto Rico or mainland US and the remaining participants were born in 19 other countries. One quarter or less of individuals born in these countries reported having a HCV risk factor. In comparison, 45% of individuals born in Puerto Rico and mainland US reported having a HCV risk factor. With each year individuals born outside the US live in the US, odds of having a risk factor increased by approximately 7% (P = 0.014). US born Hispanics are more likely to have a HCV risk factor than Hispanics born outside the US. Furthermore, the prevalence of risk factors increase among Hispanic immigrants after living in the US. These findings have a direct public health impact by providing rationale to focus HCV prevention efforts on recent immigrants.

Using methadone to treat opioid-induced hyperalgesia and refractory pain.

A patient was treated for several years with high doses of opioids for malignant pain. During a recent hospitalization, the patient's pain remained uncontrolled despite escalating doses of various opioids. We suspected that this patient suffered from the clinical phenomenon of opioid-induced hyperalgesia (OIH). The patient was then rotated from her other opioids to methadone, and her pain was adequately controlled within several days. Methadone, because of its NMDA antagonist properties, offers an effective treatment for OIH. The use of methadone for analgesia is complex and should be undertaken only by practitioners who have appropriate experience.

Hematologic problems in psychosomatic medicine.

Vitamin B12 deficiency is associated with problems in cognition, mood, psychosis, and less commonly, anxiety. Folate deficiency primarily is associated with problems in mood. Patients who have sickle cell disease, a disease of chronic pain, experience difficulties with depression, anxiety, stigma, and are at risk for substance abuse and dependence. Patients with hemophilia have benefited from advances in treatment; however, their morbidity and mortality were compounded in those who received blood products contaminated with HIV, or hepatitis B and C. Psychiatrists who practice psychosomatic medicine should expect to encounter patients with the above problems, as they are frequently seen in medical settings. Finally, most of the commonly used psychotropic medications have uncommon but potentially important hematologic side effects or may interact with the anticoagulants used in medically ill patients.

Bronchobiliary fistula successfully treated with n-butyl cyanoacrylate via a bronchial approach.

A bronchobiliary fistula is an abnormal communication between the biliary tree and the airway that can result in debilitating amounts of bilioptysis, or bile-stained sputum. The authors present an approach for the conservative management of a bronchobiliary fistula in a patient who failed traditional conservative therapy and refused surgical intervention.

Guide wire perforation leading to fatal perirenal hemorrhage from transcortical collaterals after renal artery stent placement.

After successful renal artery angioplasty and stent placement, a patient in a fully anticoagulated state developed hypotension and flank pain. Review of the intraprocedural angiogram demonstrated transcortical position of the guide wire. Computed tomography of the abdomen revealed a large perinephric hematoma. Although only a single renal artery branch was accessed with the guide wire during stent placement, subsequent emergent angiography revealed extravasation from multiple capsular branches. Renal artery embolization failed to control the hemorrhage. The patient's course rapidly deteriorated and he ultimately died. The unique angiographic finding and proposed mechanism of this fatal complication are described and discussed.

Inferior mesenteric artery embolization before endovascular aneurysm repair: technique and initial results.

PURPOSE: To report a single center's technique and initial results in the preoperative embolization of the inferior mesenteric artery (IMA) before endovascular aneurysm repair (EVAR). MATERIALS AND METHODS: Over a 3-year period, 102 patients at a single clinical site, including 86 men and 16 women aged 54-93 years (mean, 75 years), were found to have a patent IMA on computed tomographic (CT) angiography before EVAR. Coil embolization was performed after subselective catheterization with use of microcoils placed in the IMA proximal to the origin of the left colic artery. All patients in whom the IMA was visualized on flush aortography and successfully accessed underwent embolization. One month and 6 months after surgery, results in this cohort were retrospectively compared with those from a similar group of patients who underwent EVAR during the same period. These patients had patent IMAs on preoperative CT angiography but did not undergo embolization as a result of nonvisualization during flush aortography. All patients underwent EVAR with bifurcated modular devices with proximal transrenal fixation. All patients underwent postoperative follow-up with multiphase CT angiography to detect the presence of endoleak. Six-month follow-up data were available for 18 patients who underwent embolization and 54 patients who did not. Change in sac diameter was compared in these patients. RESULTS: Embolization was technically successful in 30 of 32 patients (94%) in whom it was attempted. There were no complications. At 1-month follow-up, five of 30 patients in the embolization group were noted to have a type II endoleak (17%). None of the endoleaks in this group were related to the IMA. The group with patent IMAs who did not undergo preoperative embolization had a 42% incidence of type II endoleak (P < .05). At 6 months after surgery, three of 18 patients who had undergone embolization (17%) had a type II endoleak, compared with 26 of 54 in the other group (48%; P < .05). Among the patients in whom 6-month data were available, mean changes in sac diameter were -5.2 mm (range, -24 to 2 mm) in the embolized group and -2.1 mm (range, -19 to 8 mm) in the nonembolized group. CONCLUSION: These initial results demonstrate that embolization of the IMA with subselective microcoils before EVAR is a safe and effective procedure to reduce the incidence of type II endoleaks. The data also suggest that preoperative embolization of the IMA is associated with greater shrinkage of aneurysm sac diameter at 6 months.