Sweat-gland carcinoma with neuroendocrine differentiation (SCAND): a clinicopathologic study of 13 cases with genetic analysis.

Low-grade neuroendocrine carcinoma of the skin (LGNECS) was proposed in 2017 as a new primary cutaneous neoplasm with neuroendocrine differentiation; however, it is not yet well known due to its rarity. Herein, we perform a detailed clinicopathologic analysis of 13 cases as well as panel DNA sequencing in three cases. The study included 12 males and 1 female with a median age of 71 (43-85) years. All lesions occurred on the ventral trunk. The mean tumor size was 2.2 (0.8-11.0) cm. The histopathology resembled that of well-differentiated neuroendocrine tumors (NETs) in other organs, but intraepidermal pagetoid spreading was seen in 8 (61.5%) cases and stromal mucin deposits in 4 (30.8%). Immunoreactivity for CK7, CK19, EMA, BerEP4, CEA, chromogranin A, synaptophysin, INSM1, GCDFP15, GATA3, ER, and bcl-2 were present in varying degrees in all tested cases. PTEN c.165-1G>A splice site mutation was detected by panel sequencing in one case, and GATA3 P409fs*99 and SETD2 R1708fs*4 in another case. Lymph node metastasis was seen significantly in cases with tumor size >2.0 cm [8/8 (100%) vs. 1/5 (20%)]. All three cases with size >3.0 cm were in unresectable advanced-stage [3/3 (100%) vs. 1/10 (10%)], and two of the three patients succumbed to the disease. The two cases of death revealed mild nuclear atypia (mitosis: 1/10 HPFs) and moderate nuclear atypia (2/10 HPFs). Thus, tumor size would be a better prognostic factor than nuclear atypia, mitotic count, and Ki67 index, unlike in NETs. These clinicopathologic and immunohistochemical features would represent the characteristics as skin adnexal tumors with apocrine/eccrine differentiation rather than NETs; therefore, we rename it as sweat-gland carcinoma with neuroendocrine differentiation (SCAND).

Use of laboratory testing for prediction of postoperative bleeding volume in cardiovascular surgery.

BACKGROUND: Coagulopathy and following massive bleeding are complications of cardiovascular surgery, particularly occurring after procedures requiring prolonged cardiopulmonary bypass (CPB). Reliable and rapid tests for coagulopathy are desirable for guiding transfusion. Measuring multiple coagulation parameters may prove useful. The purpose of this study is to determine the laboratory parameters predicting massive bleeding. METHODS: In a prospectively collected cohort of 48 patients undergoing cardiovascular surgery, markers of coagulation and fibrinolysis were measured using automated analyzer and their correlations with bleeding volume were determined. RESULTS: Operation time was 318 (107-654) min. CPB time was 181 (58-501) min. Bleeding volume during surgery was 2269 (174-10,607) ml. Number of transfusion units during surgery were packed red blood cells 12 (0-30) units, fresh frozen plasma 12 (0-44) units, platelets 20 (0-60) units and intraoperative autologous blood collection 669 (0-4439) ml. Post-surgery activities of coagulation factors II (FII), FV, FVII, FVIII, FIX, FX, FXI and FXII were decreased. Values of fibrinogen, antithrombin, α2 plasmin inhibitor (α2PI) and FXIII were also decreased. Values of thrombin-antithrombin complex (TAT) were increased. Values of FII, FIX, FXI and α2PI before surgery were negatively correlated with bleeding volume (FII, r = - 0.506: FIX, r = - 0.504: FXI, r = - 0.580; α2PI, r = - 0.418). Level of FIX after surgery was negatively correlated with bleeding volume (r = - 0.445) and level of TAT after surgery was positively correlated with bleeding volume (r = 0.443). CONCLUSIONS: These results suggest that several clinical and routine laboratory parameters of coagulation were individually associated with bleeding volume during cardiovascular surgery. Determining the patterns of coagulopathy may potentially help guide transfusion during cardiovascular surgery.

Spectrum of cutaneous vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): a case series.

BACKGROUND: The diverse histopathologic spectrum of cutaneous vasculitis in eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) has not been well described. METHODS: Fifteen skin biopsy specimens from 9 EGPA patients with histopathologically proven necrotizing vasculitis were reviewed clinicopathologically. RESULTS: Among 8 patients with dermal small vessel vasculitis, neutrophilic vasculitis was observed in 2 myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCA)-positive patients, whereas the remaining 6 MPO-ANCA-negative patients showed eosinophilic vasculitis in 3 and a mixed infiltrate of neutrophils and eosinophils in another 3 patients. Five patients with muscular vessel vasculitis showed vasculitis at different inflammatory stages in separate or coexisting at the same biopsied skin lesions: acute stage (eosinophilic vasculitis), granulomatous stage (granulomatous vasculitis), and healed stage. Coexistent small vessel and muscular vessel vasculitis was found in 4 patients. CONCLUSIONS: The histopathologic spectrum of dermal small vessel vasculitis in EGPA ranges from eosinophilic vasculitis with negative MPO-ANCA at one end to neutrophilic vasculitis with positive MPO-ANCA at the other end. The affected vessels ranging from dermal small vessels to subcutaneous muscular vessels in addition to the MPO-ANCA phenotype may account for the many facets of vasculitis in EGPA.

[Contribution of central hospital laboratory to critical bleeding].

It has been reported that fibrinogen products, such as fibrinogen concentrates, cryoprecipitate (CRYO), and fresh frozen plasma, are beneficial for treating coagulopathy due to massive blood transfusion. For the appropriate use of these products, it is necessary to evaluate the status of coagulopathy and determine the trigger level of the fibrinogen concentration for the administration of fibrinogen products. In our institution, we established a treatment procedure for coagulopathy due to massive transfusion in 2011. This procedure includes determination of the trigger level for administration of CRYO (150 mg/dL), timing of sample collection for the evaluation of coagulation parameters (prothrombin time, activated partial thromboplastin time, and fibrinogen) and concentration status during the operation, and a method for rapid coagulation testing (turnaround time within 15 minutes) in critical bleeding. Since 2011, we have performed 56 rapid coagulation tests for patients suffering from critical bleeding. The average turnover time was 13 minutes. According to the rapid coagulation test results, CRYO was administered to 27 patients. These results are satisfactory for treating critical bleeding patients. We stress the need for the establishment of a rapid coagulation test system in the central hospital laboratory. (Review).

[Examination of the sample centrifugation time for emergency coagulation test].

The rapidity of coagulation testing is important for use as appropriate substitution therapy in patients with, or at risk of critical bleeding requiring massive transfusion. Whereas the ordinary method of coagulation testing is known to be slow, in a critically haemorrhaging patient, a rapid turnaround time of coagulation testing becomes indispensable. To find out if coagulation test results will be affected by a shortened centrifugation time, we measured PT (prothrombin time), APTT (activated partial thromboplastin time), FIB (fibrinogen) and PLT (platelet) in plasma, using different centrifugation times (10 min, 5 min, 3 min), and analyzed the measurements. We found that, whereas centrifugation time significantly affected the PLT count in plasma (10 min; 5.17 +/- 3.71 x 10(3)/microl, 5min; 28. +/- 26.9 x 10(3)/microl, 3min; 63.7 x 10(3)/microl), PT(10min; 14.6 +/- 5.76 sec, 5min; 14.7 +/- 5.84 sec, 3min; 14.9 +/- 6.40 sec), APTT (10min; 36.4 +/- 15.9 sec, 5min; 36.8 +/- 16.5 sec, 3min; 34.7 +/- 11.4 sec) and FIB(10min; 361 +/- 134 mg/dl, 5min; 356 +/- 132 mg/dl, 3min; 356 +/- 125 mg/dl) were not affected. These data suggest that shortening centrifugation time will have no significant effect on the value of PT, APTT and FIB, in an emergency situation.

[Hemodilutional autologous blood transfusion and the roles of medical technologists as specialists of the blood transfusion].

Hemodilutional autologous blood transfusion has been experienced by 13 cases in urological surgery and has been able to save on blood, 276.6 ml in mean volume, so that this blood transfusion is thought to be valuable. And medical technologists played an important role of this method as a specialist of blood transfusion, who is expected to collaborate with surgeons as a staff.

[Metastatic malignant melanoma of the urinary bladder presenting with hematuria: a case report].

A 65-year-old man complained of macroscopic hematuria and urinary obstruction. A blood clot in the bladder eliminated transurethrally demonstrated a solitary tumor at the dome of the bladder. Transurethral resection of the bladder tumor (TUR-Bt) was performed, and histological diagnosis was malignant melanoma. Physical examination revealed a black-pigmented site on the lateral border of the right foot sole, which was dermatologically diagnosed as malignant melanoma. The vesical site was diagnosed as a metastatic melanoma of the bladder. Metastatic neoplasms of the bladder are rare, ranging from 0.1 to 6.2 percent of all bladder tumors. To our knowledge, approximately forty percent of metastatic neoplasms of the bladder originate from melanoma. Therefore, an atypical nodular lesion encountered during cystoscopy may be metastasis of malignant melanoma and dermatic lesions should be investigated.

Reticulocyte hemoglobin equivalent as a potential marker for diagnosis of iron deficiency.

Evaluation of parameters relating to serum ferritin and iron is critically important in the diagnosis of iron deficiency anemia (IDA). The recent development of automated systems for hematology analysis has made it possible to measure reticulocyte hemoglobin equivalent (RET-He), which is thought to reflect iron content in reticulocytes, in the same sample used for complete blood count tests. If RET-He is, indeed, capable of evaluating iron deficiency (ID), it would be useful for immediate diagnosis of IDA. In the present study, we examined the usefulness of RET-He for diagnosis of ID. Blood samples were obtained from 211 patients. Anemia was defined as hemoglobin (Hb) level of <12 g/dL. Iron deficiency was defined as serum ferritin level of <12 ng/mL. Patients were classified into four groups: IDA, ID, control, and non-ID with anemia. Patients in the IDA group had significantly lower RET-He levels than those in the control group. RET-He correlated with serum ferritin in the IDA and ID groups. The area under the curve for RET-He was 0.902, indicating that RET-He facilitates the diagnosis of ID with high accuracy. RET-He changed in parallel with changes in Hb during iron administration for 21 IDA patients. Our results indicate that RET-He may be a clinically useful marker for determining ID in the general population.