Lymph Node and Distant Metastases in Phyllodes Tumor of the Breast.

A 54-year-old woman who incidentally noticed a "knot" in her left breast subsequently underwent excisional biopsy which yielded a diagnosis of malignant neoplasm with sarcomatous features. Given the broad differential diagnosis and imaging findings, the patient underwent bilateral mastectomy and intraoperative sentinel lymph node assessment. An 8.5 cm tumor that was further classified as a malignant phyllodes tumor was identified in the left breast, while the frozen section interpretation came back as positive for metastatic disease which resulted in left axillary lymphadenectomy. Two months later the patient progressed to distant metastatic disease and unfortunately passed away within 6 months after her initial diagnosis. Lymph node metastasis in phyllodes tumor is an exceptionally rare event for which patients usually do not undergo intraoperative sentinel lymph node examination; however high clinical suspicion of metastatic disease would be evidence for further investigation.

Storage Artifact Masquerading as Yeast: Presenting a Diagnostic Pitfall.

Abnormal cell morphology can result from prolonged specimen storage, both for red and white blood cells. In particular, nuclear pyknosis of segmented neutrophils can occur in both peripheral blood and body fluids and may represent a diagnostic pitfall as it can mimic intracellular yeast or bacteria morphology. Pathologists are frequently asked to examine body fluid smears which are thought to contain microorganisms, and the presence of an unexpected organism can be especially concerning. Morphologic changes from prolonged storage may be encountered infrequently, and it is important to be aware of them in order to avoid misrepresentation, as additional work-up may be required for a suspected case of an unexpected fungal infection.

A comparison between liquid group A plasma and thawed group A plasma for massive transfusion activation in trauma patients.

BACKGROUND AND OBJECTIVES: The use of group A thawed 24-h plasma when resuscitating haemorrhagic shock patients has become more common; however, limited data exist on the clinical use of liquid plasma (LP). Our aim is to determine whether LP is of clinical benefit to patients requiring massive transfusion. MATERIALS AND METHODS: The objective of this retrospective study was to detect any difference in 24-h survival between patients receiving liquid or thawed plasma (TP) during their massive transfusion activation. Other objectives were to report any difference in hospital length of stay (LOS), intensive care unit (ICU) LOS and in-hospital survival. Data collected included gender, age, mechanism of injury, Injury Severity Score, Revised Trauma Score and Trauma Injury Severity Score. RESULTS: A total of 178 patients received 1283 units of LP, median 4 and range (1-56), whereas 270 patients received 2031 units of TP, median 5 and range (1-87). The two study groups were comparable in terms of gender, age, mechanism of injury, whole blood, red blood cells, platelets and cryoprecipitate transfused. The use of LP during the massive transfusion activation in traumatically injured patients was not associated with increased 24-h survival compared to when using TP, p = 0.553. CONCLUSION: Our study did not show a difference in 24-h or 30-day survival between the use of LP compared to TP in trauma patients. LP should be considered an alternative to TP in trauma patients requiring immediate plasma resuscitation.

Cutaneous iontophoresis of vasoactive medications in patients with scleroderma-associated pulmonary arterial hypertension.

BACKGROUND: It remains unknown whether the cutaneous microvascular responses are different between patients with scleroderma-associated pulmonary arterial hypertension (SSc-PAH) and SSc without pulmonary hypertension (PH). METHODS: We included 59 patients with SSc between March 2013 and September 2019. We divided patients into 4 groups: (a) no PH by right heart catheterization (RHC) (n = 8), (b) no PH by noninvasive screening tests (n = 16), (c) treatment naïve PAH (n = 16), and (d) PAH under treatment (n = 19). Microvascular studies using laser Doppler flowmetry (LDF) were done immediately after RHC or at the time of an outpatient clinic visit (group b). RESULTS: The median (IQR) age was 59 (54-68) years, and 90% were females. The responses to local thermal stimulation and postocclusive reactive hyperemia, acetylcholine, and sodium nitroprusside iontophoresis were similar among groups. The microvascular response to treprostinil was more pronounced in SSc patients without PH by screening tests (% change: 340 (214-781)) compared with SSc-PAH (naïve + treatment) (Perfusion Units (PU) % change: 153 (94-255) % [p = .01]). The response to A-350619 (a soluble guanylate cyclase (sGC) activator) was significantly higher in patients with SSc without PH by screening tests (PU % change: 168 (46-1,296)) than those with SSc-PAH (PU % change: 22 (15-57) % [p = .006]). The % change in PU with A350619 was directly associated with cardiac index and stroke volume index (R: 0.36, p = .03 and 0.39, p = .02, respectively). CONCLUSIONS: Patients with SSc-PAH have a lower cutaneous microvascular response to a prostacyclin analog treprostinil and the sGC activator A-350619 when compared with patients with SSc and no evidence of PH on screening tests, presumably due to a peripheral reduction in prostacyclin receptor expression and nitric oxide bioavailability.

A comparison between leukocyte reduced low titer whole blood vs non-leukocyte reduced low titer whole blood for massive transfusion activation.

BACKGROUND: Hemorrhagic shock is the leading cause of survivable death in trauma patients and recent literature has focused on resuscitation strategies including transfusing low-titer group O whole blood (LTOWB). Debate remains regarding whether leukocyte reduced (LR) whole blood is of clinical benefit or detriment to patients requiring massive transfusion. This study compares survival outcomes between LR-LTOWB and non-LR LTOWB. STUDY DESIGN AND METHODS: The objective of this prospective, observational study was to detect any difference in 24-hour survival between patients receiving LR-LTOWB and non-LR LTOWB during their massive transfusion activation. Secondary objectives were to report any difference in ICU LOS, ventilation days, in-hospital survival, and hospital LOS. Data collected included patient sex, age, mechanism of injury, Injury Severity Score (ISS), Trauma Injury Severity Score (TRISS), cause of death, and number of LTOWB transfused. RESULTS: A total of 167 patients received 271 LTOWB transfusions. There were 97 patients that received 168 units of LR-LTOWB while 70 patients received 103 units of non-LR LTOWB. The two study groups were comparable in terms of age, sex, ISS, TRISS, and the number of LTOWB transfused. The use of LR LTOWB during the initial massive transfusion activation in traumatically injured patients was not associated with increased 24-hour survival compared to when using non-LR LTOWB. No transfusion associated adverse events were reported. CONCLUSIONS: The administration of either LR or non-LR LTOWB was not associated with >24 hours survival in patients presenting with massive hemorrhage. The high cost and the rapid decline in platelet count of LR whole blood may be a consideration.

Mixed Venous Oxygen Saturation Is a Better Prognosticator Than Cardiac Index in Pulmonary Arterial Hypertension.

BACKGROUND: European Society of Cardiology (ESC) and European Respiratory Society (ERS) guidelines include thermodilution cardiac index (TDCI) and mixed venous oxygen saturation (SvO2) as two of the three hemodynamic determinations used in risk assessment of patients with pulmonary arterial hypertension (PAH). SvO2 may be a better measurement than TDCI to assess prognosis in patients with either idiopathic or heritable PAH. RESEARCH QUESTION: What is the concordance between TDCI and SvO2 ESC/ERS risk group allocation and their prognostic value in patients with PAH? STUDY DESIGN AND METHODS: In this retrospective study, we assessed the correlation between SvO2 and TDCI in patients with idiopathic and heritable PAH. We determined concordance in the ESC/ERS risk group allocation and association with survival, both at baseline and follow-up. RESULTS: A total of 158 patients (mean age, 58 ± 17 years; 72% women) with idiopathic (91%) and heritable (9%) PAH were included. There was moderate association between TDCI and SvO2 (r = 0.50; 95% CI, 0.37-0.62). Weighted kappa revealed a fair agreement between TDCI and SvO2 (κ = 0.30; 95% CI, 0.18-0.42), with concordance in risk group allocation in 49% of patients. During a median follow-up of 45 months (interquartile range, 23-105), 62 patients (39%) died. Using Kaplan-Meier analysis, survival was impacted by the SvO2 (log rank = 0.002) but not by the TDCI risk group allocation (log-rank = 0.51). Using the Cox proportional hazard model, adjusted for age and sex, SvO2 (but not TDCI) was associated with mortality (hazard ratio per 1% change, 0.94; 95% CI, 0.91-0.97; P < .001). INTERPRETATION: When using the cutoffs proposed by the ESC/ERS guidelines, we noted poor concordance in risk score allocation between TDCI and SvO2. In patients with idiopathic or heritable PAH, SvO2 measurements are superior to TDCI in predicting long-term mortality.

Anti-M-Induced Delayed Hemolytic Transfusion Reaction.

BACKGROUND: Anti-M is most often assumed to be naturally occurring and can be comprised of a mixture of predominantly immunoglobulin(Ig)M with a lesser IgG component. Anti-M-antibodies usually do not react at 37°C and therefore are considered to be of little clinical significance. METHODS: A 28-year-old man presented with hemorrhagic shock from numerous injuries sustained in a motor vehicle collision. The patient received several units of red blood cells (RBCs). The antibody screen, the direct antiglobulin test (DAT), and the RBC genotype were sent for laboratory evaluation. RESULTS: A total of 12 days after the first antibody screening result was negative (7 days after transfusion), the lowest hemoglobin value was 5.5 g per dL, and we observed a positive antibody screening result and DAT with immunoglobulin (Ig)G anti-M identified. After transfusion of 4 units of M antigen-negative RBC, the post-transfusion hemoglobin level increased to 8.9 g per dL. CONCLUSION: Obtaining M antigen-negative compatible RBCs is necessary in patients demonstrating IgG anti-M in plasma.

Serum Chloride Levels Track With Survival in Patients With Pulmonary Arterial Hypertension.

BACKGROUND: Serum chloride is an important homeostatic biomarker in left heart failure, with significant prognostic implications. The impact of serum chloride in the long-term survival of patients with pulmonary arterial hypertension (PAH) is unknown. We tested whether serum chloride levels are associated with long-term survival in patients with PAH. METHODS: We included patients with idiopathic or heritable PAH who had a basic metabolic panel performed at the time of their diagnostic right heart catheterization. Laboratory results were recorded both at diagnosis and 6-month follow-up. RESULTS: We included 277 patients, mean age 51 ± 18 years and 73% women, of whom 254 had a follow-up electrolyte determination at 6 months. Serum chloride was 102.9 ± 3.9 mM/L at diagnosis. A serum chloride ≤ 100 mM/L was noted in 65 (24%) and 53 (21%) patients at diagnosis and 6 months, respectively. Patients with serum chloride ≤ 100 mM/L at 6 months tracked with increase mortality when adjusted by age, sex, pulmonary vascular resistance, diuretics or prostacyclin analogs usage, and serum creatinine and sodium at 6 months (hazard ratio, 1.83; 95% CI, 1.11-3.00). This group of patients was older, with decreased functional capacity, had worse renal function, took more diuretics, had higher pulmonary artery wedge pressure but lower mean pulmonary artery pressure, transpulmonary gradient, and pulmonary vascular resistance. CONCLUSIONS: Serum chloride at 6 months from the PAH diagnosis is a strong and independent predictor of mortality in patients with idiopathic or heritable PAH, even after adjusting serum sodium, renal function, diuretic, and prostacyclin analog usage.

Hypoxemia in patients with idiopathic or heritable pulmonary arterial hypertension.

BACKGROUND: The prevalence and prognostic implications of hypoxemia either at rest or during six-minute walk test (6MWT) in patients with idiopathic or heritable pulmonary arterial hypertension (IPAH or HPAH) have not been systemically studied. OBJECTIVES: We sought to determine the prevalence, phenotypic and prognostic implications of hypoxemia in patients with IPAH and HPAH. METHODS: Patients with IPAH or HPAH were identified from the Cleveland Clinic Pulmonary Hypertension Registry. Pulse oximetry (SpO2) at rest and during 6MWT was used to define hypoxemia at rest or during activities when measurements were lower than 90%, respectively. RESULTS: A total of 292 patients (age 50.6 ± 18.0 years, 73% females) with IPAH (88%) and HPAH (12%) were included. Of them, 143 (49%) had SpO2 >90% at rest and during 6MWT, 89 (31%) subjects had hypoxemia during 6MWT and 60 (20%) had hypoxemia at rest. Patients with hypoxemia had older age, greater body mass index, higher prevalence of cardiovascular risk factors, worse functional capacity and pulmonary function tests but less severe pre-capillary pulmonary hypertension. Individuals with hypoxemia either at rest or during the initial 6MWT had worse long-term survival when compared to subjects without hypoxemia, even when adjusting for a great number of potential confounders. (HR: 2.5 (95% CI: 1.54-3.98)). CONCLUSIONS: Hypoxemia in patients with IPAH and HPAH is associated with more comorbidities, less severe pre-capillary pulmonary hypertension and worse survival.

Assessing the kinetics of microbubble appearance in cirrhotic patients using transthoracic saline contrast-enhanced echocardiography.

BACKGROUND: The number of cardiac cycles that need to be reviewed by echocardiography before a significant intrapulmonary shunt can be excluded remains unclear. METHODS: We retrospectively identified patients with cirrhosis who underwent technetium-99 m-labeled macroaggregated albumin scanning. The kinetics of bubble appearance after the injection of agitated saline during transthoracic echocardiograms were assessed in these patients. RESULTS: For the 64 eligible patients, the mean ± SD age was 56 ± 9 years. The median (IQR) shunt fraction by radionuclide scanning was 7.7% (2.8%-19.9%). Microbubbles were seen in the left atrium (LA) and left ventricle (LV) at a median (IQR) of 4 (2-5) and 4 (2-5) beats, respectively. The number of heart cycles before microbubbles appeared in the LA or LV was inversely associated with the nuclear scanning shunt fraction (R = -0.42, P = .001, R = -0.46, P < .001, respectively). If no microbubbles were detected by heart cycle 7, the shunt fraction was uniformly less than 3%. Patients with arterial oxygen <60 mm Hg, compared to ≥60 mm Hg, had earlier appearance of microbubbles in the left heart chambers (2.6 ± 1.9 vs 4.0 ± 2.3 beats, P = .046). CONCLUSIONS: In patients with advanced cirrhosis suspected of having hepatopulmonary syndrome, a greater shunt fraction during nuclear scanning was associated with more pronounced hypoxemia and a prompt and more intense appearance of microbubbles in the left-sided heart chambers. Patients with a shunt fraction above 3% have microbubbles in the LA or LV at some point during the first seven heart cycles.