Cardiac Amyloidosis in the Setting of a Sarcomatous Pericardial Mass.

Cardiac amyloidosis is a significantly underdiagnosed disease but should be suspected in anyone with restrictive heart physiology. Here, we present a case of a sarcomatous pericardial mass confounding the patient's progressive diastolic heart failure. Amyloidosis was eventually discovered by piecing together serial transthoracic echocardiogram, functional MRI, and technetium-99m (99mTc) pyrophosphate scintigraphy findings along with a negative lab workup. The presence of the sarcomatous pericardial mass raised the question of whether it played a role in the onset and progression of amyloidosis, but nonetheless, the presence of both diseases rendered multifaceted challenges regarding our patient's care. Anyone suspected to have amyloidosis should receive appropriate testing for a definitive diagnosis to catch the disease process and offer early treatment, as exciting research is emerging showing transthyretin stabilizers to have a reduction in all-cause mortality.

The 13-year bleed: Exuberant amyloid angiopathies, angiodysplasias, and acquired coagulopathies of the gut.

Amyloidosis is a disorder characterized by extracellular deposits of proteins that are prone to aggregate and form insoluble fibrils. Amyloid deposits limited to a single organ or tissue without the involvement of any other site in the body is uncommon. We report a 75-year-old man with previously treated non-Hodgkin's lymphoma who presented with recurrent gastrointestinal hemorrhage. Histopathology showed amyloid deposition within vascular malformations. His bleeding continued with the cause rooted in the fundamental building blocks-clotting factors. We discuss the interplay of the pathophysiology of lymphoma, amyloidosis, and factor X deficiency in a patient with preexisting angiodysplasias leading to refractory gastrointestinal bleeding. To our knowledge, there are only 3 reported cases of concomitant amyloidosis and angiodysplasia in the colon, and none involving the small bowel.

Oriental Cholangiohepatitis in a Caucasian male.

Recurrent pyogenic cholangitis (RPC) is a condition found almost exclusively in individuals who lived in Southeast Asia. We report a case of a Caucasian veteran diagnosed with RPC after presenting with a 5-year history of recurrent fevers and abdominal pain 20 years after serving in Japan, South Korea, and Guam. Extensive evaluation led to the diagnosis of RPC with improvement after biliary decompression and antibiotics. Although rare, RPC should be considered in individuals who present with recurrent bouts of abdominal pain and fevers regardless of race.

Response to pegylated interferon in a COVID-19-positive elderly woman with primary myelofibrosis treated with ruxolitinib.

An 83-year-old female had asymptomatic SARS-CoV-2 infection while taking ruxolitinib. She remained RT-PCR positive for viral RNA for >120 days, and Pegylated interferon for 4 weeks led to viral RNA clearance. The observations support combination therapy of ruxolitinib + interferon for COVID-19.

Hiccups: You got to be kidney me!

Hiccups are usually benign processes that most people experience with spontaneous resolution. However, persistent hiccups with a duration greater than 48 h may prompt a thorough workup for structural, infectious, or inflammatory disorders. Moreover, toxic-metabolic states, including renal dysfunction and/or uremia, have also been identified as sources of hiccups. We present a 62-year-old male with persistent hiccups that developed acute, worsening renal function. Workup for a possible intrinsic renal disease process was negative. Ultimately, a kidney biopsy was performed, demonstrating acute tubular injury with oxalate crystals and interstitial fibrosis. Dialysis was initiated with resolution of the hiccups, and kidney function improved over an extended period of time.

Demyelinating syndrome in systemic sclerosis and neuromyelitis optica.

BACKGROUND: This article reports a case diagnosis of a 44-year-old female who presented with intractable hiccups and vomit complicated with an acute onset of paraplegia. Transverse myelitis was evident on MRI and serological studies were consistent with Neuromyelitis Optica (NMO) based on NMO-IgG sero-positivity. Further studies revealed positive ANA, anti-RNA polymerase III autoantibodies, and Scl-70, leading to a concurrent diagnosis of systemic sclerosis (SSc). The coexistence of these two disease processes and their underlying clinical manifestations and therapeutic interventions are seldom reported in literature and are worth reporting. CASE PRESENTATION: The patient was treated with high dose steroids, and subsequently developed malignant hypertension and acute renal failure, later identified on biopsy as steroids-induced scleroderma renal crisis. Although Neuromyelitis Optica spectrum disorder (NMOSD) has often been associated with various collagen and autoimmune diseases, the coexistence of NMOSD and SSc presented a challenge where the patient underwent aggressive physical therapy and necessitated an intervention with Rituximab to achieve an appropriate clinical response. We have received a written consent forms from the participant in our study, and we have them on file in case they are requested. We have also received the patient's written consent for the data and images presented in this article. CONCLUSION: This article expands on NMOSD associated autoimmune diseases. Systemic Sclerosis is an insidious disease that is often diagnosed late as not all patients often report skin manifestation. The finding suggests that patients presenting with acute neurological manifestations get tested for NMO-IgG/AQP-4 antibodies and other immunological studies based on clinical findings.

Reference ranges of lymphocyte subsets in healthy adults and adolescents with special mention of T cell maturation subsets in adults of South Florida.

BACKGROUND: Analysis of peripheral blood lymphocyte subsets has become an essential tool in the evaluation of outcome of diagnostic and research related questions in immunological and pathological conditions. Periodic evaluation and establishment of normal lymphocyte reference ranges are required in clinical and research settings of various immunodeficiency disorders for evaluation of the significance of observations. It is also important that age and gender specific lymphocyte subset reference ranges should be locally established for meaningful comparison and accurate result interpretation as age plays a significant role in the development of immune system. METHODS: We performed dual platform flow cytometry to determine reference ranges for lymphocyte subsets (CD3, CD4, CD8, CD19 [B cells] and CD16+CD56+ [Natural Killer - NK cells]) in 50 adolescents (age range: 12-18) and 100 adults (age range: 21-67) along with T cell maturation, activation and co-stimulatory molecules in healthy multiracial adult population of South Florida. RESULTS: The lymphocyte reference ranges percentages [absolute counts - Abs, cells/µl] unadjusted for gender differences for adolescents are: CD3: 49-83 [939-2959]; CD4: 27-53 [467-1563]; CD8: 16-40 [259-1262]; CD19+ B cells: 8-31 [169-1297] and CD16+CD56+ NK cells: 3-30 [59-1178] and for adults are: CD3: 65-88 [983-3572]; CD4: 26-62 [491-2000]; CD8: 14-44 [314-2,087]; CD19+ B cells: 2-27 [64-800] and CD16+CD56+ NK cells: 2-27 [27-693]. The ranges for CD4:CD8 ratio for adolescents and adults are 0.7-2.6 and 0.6-4.4, respectively. Gender based analysis of relative percentages of lymphocyte subsets showed no significant differences between adult and adolescent males and females. The mean CD4:CD8 ratio was significantly higher in adult females than males (P=0.04) and in adolescents this difference was not significant between genders. The mean CD3 and CD4 T cell percentages were higher and CD19 cell percentages were lower in adults compared to adolescents (P<0.0001). Absolute lymphocyte counts showed a positive correlation with the absolute counts of CD3+, CD4+, CD8+, CD19+, CD16+CD56+, CD45RO+ and CD45RA+ cells (all correlations with P<0.0001 except CD45RO [P=0.01] and CD45RA [P=0.03]). CONCLUSION: The reference values of peripheral blood lymphocyte subsets were analyzed in healthy adolescent and adult population of South Florida. This study indicates the need for periodic evaluation and establishment of lymphocyte reference ranges for patient population served based on gender and age since these could influence immune status and treatment outcome.

Disparities in HIV-treatment responses between Haitians, African Americans, and Hispanics living in Miami-Dade County, Florida.

BACKGROUND: Studies evaluating response to treatment with Highly Active Antiretroviral Therapy (HAART) fail to examine Haitian patients living in the U.S. as a distinct group. METHODS: This study was designed to determine the effectiveness of HAART in Haitians compared with other minority groups. We conducted a retrospective cohort study of HIV patients from two clinics. The cohort included 96 Hispanics, 60 African Americans, and 49 Haitians, after reviewing a total of 891 charts. RESULTS: At 96 weeks, fewer Haitians (58.5%) achieved a suppressed viral load than African American (74.1%) or Hispanic (82.8%) patients (p=.021). Median CD4 counts at baseline were lowest among Haitians, with 158 cells/mm3, compared with African Americans, 176 cells/mm3 and Hispanics, 199 cells/mm3. CONCLUSIONS: Haitians are not doing as well on HAART as other groups. This may be explained by linguistic, cultural, or other barriers that are not currently addressed by the health care system in the United States.