Recurrent intracranial bleed in a child receiving prophylaxis with emicizumab

Haemophilia ◽  
2021 ◽  
Author(s):  
Hong Kai Wesley Teo ◽  
Wan Hui Wong ◽  
Joyce Ching Mei Lam
Keyword(s):  
Author(s):  
Sunil V. Furtado ◽  
Pranoy Hegde ◽  
Rasmi Palassery ◽  
B. P. Karunakara

AbstractFactor XIII (FXIII) deficiency is a rare bleeding disorder with affected patients having high propensity for intracranial hemorrhage. A 12-year-old girl presented with severe headache, limb weakness, and rapidly worsening sensorium over 4 days. Magnetic resonance imaging of the brain and computed tomography (CT) of the head showed intraparenchymal bleed. Patient had normal coagulation profile and abnormal FXIII level. The perioperative management included cryoprecipitate transfusion to bring the FXIII value to 74%. She underwent craniotomy and evacuation of the hematoma. Postoperatively, she received prophylaxis against rebleed with cryoprecipitate. In the absence of FXIII concentrate, correction of FXIII deficiency is possible with cryoprecipitate in emergent situations.


2017 ◽  
Vol 37 (1) ◽  
pp. 104-107 ◽  
Author(s):  
Sudhir Adhikari ◽  
Eva Gauchan ◽  
Tejesh Malla ◽  
Brijesh Sathian ◽  
Kalipatanam Seshagiri Rao

Vitamin K deficiency bleeding (VKDB) can manifest as intracranial hemorrhage (ICH) and is still prevalent in poor resource countries. Infants aged one to twelve months with the diagnosis of ICH from 1st July 2011 to 30th June 2016 were included. There were 16 cases of ICH attributed to vitamin K deficiency. Clinical presentations were anemia16 (100%), bulged fontanel 13(81.3%), seizures 10(62.5%), vomiting 8(50%) and fever 9(56.3%). Mean INR at admission was 8.575±7.267 and 1.868±0.838 after three doses of vitamin K administration. Sites of intracranial bleed were parenchymal 5(31.3%), subdural 4(25%), extradural 2(12.5%), ventricular 2(12.5%). In 3(18.8%) of cases bleeding was more extensive involving more than one site. Mortality was 4(25%) and 3(18.8%) had abnormal neurological findings at discharge. There is an urgent need for national policy for vitamin K prophylaxis at birth.


Author(s):  
Shekhar Singh Jadaun ◽  
Sanjiv Saigal ◽  
Ana Hasnain ◽  
Mukesh Kumar Olaniya ◽  
Dhiraj Agrawal ◽  
...  

2020 ◽  
Vol 10 (3) ◽  
pp. 92-96
Author(s):  
Deepak Madhavi ◽  
Shamama Subuhi ◽  
Mohammed Zubai

Thrombocytopenia is one of the commonest haematological disorders in the neonatal period, affecting up to a third of those admitted to neonatal intensive care units. It is well recognized that many fetomaternal and neonatal conditions are associated with thrombocytopenia. The majority of episodes of neonatal thrombocytopenia are relatively mild, self-limiting and of short duration but it may cause severe morbidity & mortality due to severe complication like IVH. Methods & material: 140 Newborn admitted in tertiary care NICU were selected to find out outcome and etiology of neonatal thrombocytopenia. Detail maternal history and neonatal physical examination done and Neonates were followed for outcome, relevant investigation done according to cases. Result: Out of 140 neonates 63 neonates had thrombocytopenia (45%).42.8% neonates were premature out of which 63.3% had thrombocytopenia. Other neonatal risk factor for thrombocytopenia are sepsis 38 (74.5%), SGA/IUGR 28(80%) and NEC 9(100%). Maternal risk factor for thrombocytopenia are eclampsia81.8% and infection during pregnancy 72.72%. 95.5 % of all study population were discharged.4.5 % cases of whole study population didn’t survive. 4.54% of mild, 9.09% of moderate and 60 % of severe thrombocytopenic babies didn’t survive. Conclusion: Bleeding manifestations i.e. mucosal, cutaneous and intracranial bleed were significantly associated with severe thrombocytopenia. 60% of mortality was found in severe thrombocytopenic group. Thus, severe thrombocytopenia was found to be a predictor of poor outcome in sick neonates of NICU.


2001 ◽  
Vol 80 (8) ◽  
pp. 766-767 ◽  
Author(s):  
Rashmi Bagga ◽  
Harjeet Sawhney ◽  
Shilpi V. Saxena ◽  
Neelam Aggarwal ◽  
Kala Vasishta

2003 ◽  
Vol 12 (1) ◽  
pp. 83-90 ◽  
Author(s):  
Helen Newman ◽  
Jo Anna Reems ◽  
Theodore H. Rigley ◽  
Daniel Bravo ◽  
D. Michael Strong

The purpose of this retrospective analysis was to determine whether there were donor factors that were useful for predicting the yield of nucleated cells from marrow derived from cadaveric vertebral bodies. An analysis of 132 donors over a 6-year period was performed. The average number of vertebral bodies procured from each donor was 10.2 ± 1.6 (range 5–14). The total number of nucleated cells recovered per donor ranged from 24 × 109 to 160 × 109 with an average recovery of 69 ± 28 × 109 cells. The cell viability of the recovered cells was >95%. The average age of the donors was 33 ± 14 years (mean ± SD; range 12–65) with an average weight of 169 ± 41 lb (range 82–308 lb). Males comprised 68% of the donor population. The average number of days from admission to death was 1.9 ± 1.7 with a range of 1–11.4 days and the interval between asystole and procurement averaged 3.1 ± 2.3 h (range (0.1–14.7 h). The majority of donors died from head trauma due to an intracranial bleed, gunshot wound, or closed head injury. Regression analysis of the data indicated that the total nucleated cell yield tended to decrease with increasing time between hospital admission and death. The data also indicated that in general female donors yielded lower cell numbers independent of age and male donors under 30 years of age yielded the highest number of cells.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4903-4903
Author(s):  
Gautam Borthakur ◽  
Hagop Kantarjian ◽  
Elihu Estey ◽  
Srdan Verstovsek ◽  
Guillermo Garcia-Manero ◽  
...  

Abstract Homoharringtonine (HHT) is a plant alkaloid that inhibits protein synthesis, induces differentiation and apoptosis of myeloid cells. We initiated a phase II study of intra-venous HHT in patients (pts) diagnosed with MDS by WHO criteria to assess safety and efficacy of HHT in this setting. Responses were recorded according to the MDS response criteria defined by NCI working group. Pts received HHT 2.5 mg/m2 by continuous 24-hour intravenous infusion daily for 7 days every 4 weeks until a) a complete remission (CR) was achieved, or b) failure to achieve a CR after 3 courses of therapy or c) unacceptable toxicities developed. The dose was increased to 3.0 mg/m2 for pts with no response after the 1st course. Nine pts have been enrolled (6 male and 3 female). Median age was 70 yrs (range 55 to 84 yrs) and 4 pts had secondary MDS. Four pts had refractory anemia with excess blasts-1 (RAEB-1), 3 had RAEB-2, 1 had chronic myelomonocytic leukemia (CMML-1) and 1 had MDS/myeloproliferative disorder (MDS/MPD). IPSS score was intermediate-1 in 1 pt, intermediate-2 in 7 pts and high in 1 pt. Seven pts (78%) received only one cycle of HHT. One (11%) pt had a CR (including cytogenetic CR) that lasted for 67 days, 5 pts discontinued treatment due to lack of response and1 pt with stable disease decided to withdraw from the study. After a median follow-up of 54 days (range, 37 to 145 days) 2 pts have died, one on day 37 due to intracranial bleed secondary to thrombocytopenia and one on day 39 due to invasive fungal infection. Grade 2 toxicities included fatigue (4 pts), diarrhea (3 pts) and nausea (3 pts). Grade 3 cytopenias were seen in all pts and grade 3 nausea in 1 pt. Three pts had neutropenic fever. Treatment during first course was interrupted after 5 days of infusion in 1 pt due to grade 3 congestive heart failure (CHF). This pt received his second course of treatment without recurrence of CHF. In conclusion, HHT has modest activity in intermediate to high risk MDS. After first interim analysis, the study meets criteria for continued accrual.


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