14 research outputs found
Myeloid Sarcoma of the Skin in a Patient with Myelodysplastic Syndrome
ABSTRACT We report the case of a 76-year-old woman who presented with asymptomatic extensive erythematous. Firm plaques were noted over the right cheek. Complete blood count was normal, as was a peripheral smear. An excision biopsy taken from the cheek showed infiltration of the dermis and hypodermis with atypical cells which were strongly positive for human leukocyte antigen (HLA-DR) and lysozyme and were moderately myeloperoxidase (MPO) enzyme. The results of immunohistochemical staining for CD34, CD117, CD3, CD4, CD8, CD20, CD23, CD56, and ALK-1 were negative. Bone marrow analysis indicated myelodysplastic syndrome RAEB 1 while cytogenetic finding showed tetrasomy 8. It was recommended that the patient undergo local radiotherapy of skin lesions, but she refused and was lost to follow-up. KEY WORDS: skin; myeloid sarcoma; myelodysplastic syndrome </p
Myeloid Sarcoma of the Skin in a Patient with Myelodysplastic Syndrome
ABSTRACT We report the case of a 76-year-old woman who presented with asymptomatic extensive erythematous. Firm plaques were noted over the right cheek. Complete blood count was normal, as was a peripheral smear. An excision biopsy taken from the cheek showed infiltration of the dermis and hypodermis with atypical cells which were strongly positive for human leukocyte antigen (HLA-DR) and lysozyme and were moderately myeloperoxidase (MPO) enzyme. The results of immunohistochemical staining for CD34, CD117, CD3, CD4, CD8, CD20, CD23, CD56, and ALK-1 were negative. Bone marrow analysis indicated myelodysplastic syndrome RAEB 1 while cytogenetic finding showed tetrasomy 8. It was recommended that the patient undergo local radiotherapy of skin lesions, but she refused and was lost to follow-up. KEY WORDS: skin; myeloid sarcoma; myelodysplastic syndrome </p
Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: Report of two cases
Introduction. Bernard-Soulier syndrome (BSS) is a rare inherited bleeding
disorder characterized by giant platelets thrombocytopenia e prolonged
bleeding timee frequent hemorrhages with considerable morbidity. Data on the
outcome of pregnancy and gynecological intervention in BSS are rare and there
are no general therapeutic recommendations. Cases Outline. We report two
cases of BSS. In the first case a 29-year-old patient with BSS was admitted
in 8 weeks of gestation. The diagnosis of BSS was made on the basis of
prolonged bleeding time, giant-platelets thrombocytopenia, and absent
ristocetin-induced platelet aggregation. In 38 week of gestation Cesarean
section, with platelets transfusion preparation, was performed. Obstetric
intervention passed without complication. Postoperative course was
complicated with a three-week vaginal bleeding resistant to platelet
transfusion. Neonate platelet count was normal. Our second case was a
28-year-old patient with BSS, hospitalized for ovarial tumor surgery. The
patient was prepared for the intervention with platelets transfusion. The
surgery was uncomplicated, but on the second postoperative day a massive
vaginal bleeding, resistant to the platelet transfusion, developed. Bleeding
control was achieved with activated recombinant factor VII. Twelve hours the
patient developed later hypertensive crisis with epileptic seizure due to
subarachnoid hemorrhage. Therapy was continued with platelet transfusion,
antihypertensive and antiedema drugs. PH examination of tumor tissue showed
hemorrhagic ovarial cyst. Conclusion. Obstretic and gynecological
intervention in women with BSS may be associated with a life-threatening
bleeding thus requiring a multidisciplinary approach with adequate
preparation. Because of the limited data in the literature, it is not
possible to provide firm management recommendations and each case should be
managed individually
Procarboxypeptidase U (TAFI) contributes to the risk of thrombosis in patients with hereditary thrombophilia
Introduction: It is considered that high plasma levels of procarboxypeptidase U (proCPU or TAFI) can promote the development of thrombosis, but data comparing proCPU levels in thrombophilia carriers and healthy subjects are rather scarce. Moreover, the results of previous studies on the risk of thrombosis related to high proCPU concentration in this population were not consistent. Although the 325 polymorphism, of proCPU has a significant effect on the CPU half-life, it's influence on the risk of thrombosis or spontaneous pregnancy loss in carriers of hereditary thrombophilia is not clear. Materials and Methods: The study population consisted of 144 thrombophilic patients (94 heterozygous and 10 homozygous carriers of FV Leiden, 26 heterozygous carriers of the prothrombin G20210A variation and 14 double carriers of FV Leiden and FII variation) and 69 healthy controls. Results: The results show that patients with inherited thrombophilia have a tendency toward lower mean proCPU plasma levels compared to healthy controls, however, this difference was only significant in carriers of FII G20210A (p = 0.014). A higher frequency of the most stable Ile325Ile proCPU was seen among carriers of I'll G20210A mutation compared to the control group (19% vs 7%; p = 0.186). In the second part of the study proCPU as a risk factor for thrombosis was evaluated. In heterozygous carriers of FV Leiden or FII G20210A high levels of proCPU conferred to an almost 4-fold increased risk for spontaneous onset thrombosis. The more stable Ile325Ile proCPU seems to impose a higher risk for clinical manifestation of the thrombophilic condition. Finally, a significant positive correlation between F1 + 2 and proCPU concentration was seen. Conclusion: The increased risk of thrombosis in thrombophilia patients is not only ascribable to an increased thrombin generation, but also high levels of proCPU and the presence of the 325Ile genotype tip the balance towards thrombotic tendency even further
Correlation to FVIII : C in two thrombin generation tests: TGA-CAT and INNOVANCE ETP
Introduction: Several thrombin-generation tests are available, but few have been directly compared. Our primary aim was to investigate the correlation of two thrombin generation tests, thrombin generation assay-calibrated automated thrombogram (TGA-CAT) and INNOVANCE ETP, to factor VIII levels (FVIII:C) in a group of patients with hemophilia A. The secondary aim was to investigate inter-laboratory variation for the TGA-CAT method. Methods: Blood samples were taken from 45 patients with mild, moderate and severe hemophilia A. The TGA-CAT method was performed at both centers while the INNOVANCE ETP was only performed at the Stockholm center. Correlation between parameters was evaluated using Spearman's rank correlation test. For determination of the TGA-CAT inter-laboratory variability, Bland-Altman plots were used. Results: The correlation for the INNOVANCE ETP and TGA-CAT methods with FVIII:C in persons with hemophilia (PWH) was r=0.701 and r=0.734 respectively. The correlation between the two methods was r=0.546. When dividing the study material into disease severity groups (mild, moderate and severe) based on FVIII levels, both methods fail to discriminate between them. The variability of the TGA-CAT results performed at the two centers was reduced after normalization; before normalization, 29% of values showed less than ±10% difference while after normalization the number increased to 41%. Conclusions: Both methods correlate in an equal manner to FVIII:C in PWH but show a poor correlation with each other. The level of agreement for the TGA-CAT method was poor though slightly improved after normalization of data. Further improvement of standardization of these methods is warranted