• Neonatal Medicine
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• v.18 no.1
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• pp.14-22
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• 2011

Neonatal bleeding is a common problem encountered in nursery rooms or neonatal intensive care units, especially among premature infants. Furthermore, owing to recent remarkable improvement of neonatology, survival rates of preterm neonates have increased; hence, neonatal bleeding cannot be emphasized enough. Since the total blood volume of neonates is small, bleeding can be one of the causes of morbidities and mortalities. Therefore, rapid diagnosis and immediate therapy is urgently needed. The patient's medical history including a familial history of a bleeding disorder or of a previously affected infant who suffered from bleeding along with maternal and neonatal drugs can provide important diagnostic clues. Presence of bleeding with or without petechiae and ecchymoses in a healthy term or late preterm infant with thrombocytopenia but normal prothrombin time and activated partial thromboplastin time strongly suggests a congenital bleeding disorder. For a sick infant who is bleeding from multiple sites, an acquired disorder such as disseminated intravascular coagulation is suspected. Intracranial hemorrhage in term or late preterm infants without a history of birth trauma is highly suggestive of coagulation disorders. The purpose of this review is to summarize recent advances in diagnostic methods is as well as basic concepts of neonatal hemostatic disorders. First, an outline of background information will be presented followed by a discussion of primary and secondary hemostatic disorders as well as inherited and acquired disorders.

• Korean Journal of Clinical Laboratory Science
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• v.54 no.2
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• pp.163-166
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• 2022

Acquired hemophilia A (AHA) is an uncommon autoimmune bleeding disorder in which autoantibodies that affect the functions of factor VIII (FVIII) are present in the blood. The initial diagnosis of AHA is difficult as the presentations of AHA differ from those of congenital hemophilia A. Moreover, the treatment of AHA is more complex due to the presence of autoantibodies against FVIII. Here, we present a case report of postpartum AHA, to increase the perception and knowledge regarding the recognition and management of such cases. We present a young female with the chief complaint of vaginal bleeding and upper arm ecchymosis. Laboratory results exhibited isolated prolonged activated partial thromboplastin time (APTT) and FVIII inhibitors. The patient was treated with corticosteroids, FVIII concentrates, and a bypassing agent. In conclusion, unexplained postpartum bleeding, unmanageable with basic hemostatic measures, should lead to clinical suspicion of an acquired bleeding disease.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.3
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• pp.1535-1537
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• 2016

Background: Primary myelodysplastic syndrome (MDS) is an acquired clonal disorder of myeloid progenitor cells, characterized by peripheral cytopenias in the presence of hypercellular marrow with dysplastic features. Our aim was to study the demographical and clinicopathological features of adult Pakistani patients with MDS at disease presentation. Materials and Methods: This single centre study was conducted at Liaquat National Hospital and Medical College, extending from January 2010 to December 2014. Data were retrieved from the patient archives. Results: Overall 45 patients were diagnosed at our institution with de novo MDS during the study period. There were 28 males and 17 females. Age ranged between 18 and 95 years with a mean age of $57.6{\pm}17.4years$ and median of 64 years. The male to female ratio was 1.7:1. The main presenting complaints were generalized fatigue (60%), fever (33.3%), dyspnea (15.5%), bleeding (13.3%) and weight loss (11.1%). Examination was unremarkable in 42.2% of patients. Physical examination revealed pallor in 37.7%, followed by petechial and purpuric rashes in 20%. The commonest laboratory finding was anemia (hemoglobin < 10 g/dl in 41 (91.1%) patients. Out of these, 27 (60%) patients had normocytic anemia, followed by macrocytic (22.2%) and microcytic (8.8%). Conclusions: Primary MDS in Pakistani patients demonstrates a male preponderance. The proportion of anemic patients was high in our series with predominance of normocytic anemia. However, other clinico-hematological features appear comparable to published data.

• Neonatal Medicine
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• v.17 no.1
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• pp.132-135
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• 2010

Although the majority of abnormal bleeding during the neonatal period results from acquired coagulation disorders, inherited coagulation disorders can also manifest at this time. Hemophilia is the most common of inherited coagulation disorder. Although 40-70% of cases with hemophilia are diagnosed in the neonatal period, few cases have been reported in premature infants. We report a case of a premature infant born at 31 weeks of gestation, diagnosed with hemophilia A by blood coagulation test, coagulation factor assay and study of the F8 gene. The baby was treated with recombinant factor VIII (Recombinate$^{(R)}$, USA) because of repeated seizures and intramuscular hematoma.

• Journal of the korean academy of Pediatric Dentistry
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• v.23 no.3
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• pp.593-600
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• 1996

This case report is a treatment of patient with hemophilia A and autism. The patient's chief complaint was treatment of dental caries on entire dentition and he has an impacted mesiodens located on the apex of the upper right primary central incisor. The patient was consulted with pediatrician and anesthetist about the detailed discussion of the complexities of hemorrhagic disorder. Because he had some problems of behavior management and bleeding, the treatment was done under the gerneral anesthesia. The following results were obtained. 1. Consult with the patient's physician and hematologist about the replacement therapy and bleeding tendency. 2. For the severe hemophiliac child who requires extensive or surgical treatment, general anesthesia may be indicated for the comprehensive care. 3. For the hemophiliac child who has a behavior management problem due to autism and other defects, general anesthesia may be considerable. 4. Care must be taken during dental procedures not to causing a bleeding. 5. Local hemostatic methods must be acquired for the emergency state. 6. Neve prescribe aspirin or non-steroidal anti-inflammatory drugs such as ibuprofen, indomethacin, and phenylbutazone. These drugs affect platelet aggregation and exaggerate the bleeding defect. 7. Do not be afraid of hemophiliac patient, and never compromise quality of dental care.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.1
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• pp.357-360
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• 2016

Background: Acute myeloid leukemia (AML) is an acquired clonal frequent malignant disorder of myeloid progenitor cells. Our aim was to study demographical and clinicopathological features of adult Pakistani AML patients at presentation. Materials and Methods: In this single centre study extending from January 2010 to December 2014, data were retrieved from the patient records with a predetermined performa and analyzed with SPSS version 22. Results: Overall 125 patients were diagnosed at our institution with de novo AML during the study period. There were 76 males and 49 females (ratio 1.5:1), with an age range between 15 and 85 years and a mean age of $38.8{\pm}20.1years$. The major complaints were fever (72.8%), generalized weakness (60%), bleeding (37.6%) and dyspnea (12%). Physical examination revealed pallor in 56.8%, splenomegaly and hepatomegaly in 16% and 12.8%, respectively, and lymphodenopathy in 10.4%. The mean hemoglobin was $8.19{\pm}2.12g/dl$ with a mean MCV of $86.0{\pm}9.83fl$, a mean total leukocyte count of $43.1{\pm}68.5{\times}10^9/l$, an ANC of $3.09{\pm}6.66{\times}10^9/l$ and a mean platelet count of $62.3{\pm}78.6{\times}10^9/l$. Conclusions: AML in Pakistani patients is seen in a relatively very young population with male preponderance, compared with the west. However, clinico-pathological features appear comparable to published data.