Five patients with severe haemophilia A and high responding inhibitors underwent laparoscopic or open surgery on the digestive tract (appendicectomy, cholecystectomy, partial colectomy, or haemorrhoidectomy) with recombinant activated factor VII (rFVIIa) prophylaxis. rFVIIa was administered at a dose of 92-127 mug/kg prior to surgery and then every 2 h for 18-56 h before increasing the dosing interval. One patient was switched to a continuous infusion after 48 h of rFVIIa boluses. rFVIIa treatment lasted between 5 and 14 days in four patients, with good or excellent efficacy (total dose, 3.13-9.28 mg/kg). The fifth patient, who underwent surgery for prolapsed haemorrhoids, bled on day 6 and day 10 after the procedure, despite a satisfactory prothrombin time and factor VII coagulant level. The rFVIIa dose regimen was increased after the second bleeding episode, then the bleeding rapidly ceased after this modification to the treatment regimen. The total dose of rFVIIa used was 12.65 mg/kg, and treatment lasted 17 days. Antifibrinolytic treatment was used concomitantly in all five patients. Clinical and biological tolerability was excellent, and no increase in the anti-factor VIII inhibitor titre was observed. These results suggest that rFVIIa prophylaxis is effective in haemophilia A patients with factor VIII inhibitors who are undergoing elective or emergency intestinal surgery. Further studies are required to optimize the dose regimen and treatment period according to the surgical indication and technique.

Background The combination of visceral leishmaniasis (VL) and macrophage activation syndrome (MAS) makes the diagnosis difficult due to their similar clinical presentation, with a poor prognosis especially since the treatment is still poorly codified. We report the case of a 17-month-old female patient from Berkane, presenting for a 3 months history of anarchic fever with anemic syndrome made up of pallor and hemorrhagic syndrome made up of epistaxis. Physical examination revealed a temperature of 39 ° C, lower limbsedema, paleness of skin and mucous membranes, gingival petechiae, bleached hair, and hepatosplenomegaly. Case presentation The complete blood count showed pancytopenia with deep aregenerative normochromic normocytic anemia at 3 g/dL, leukocytes were at 4860/mm 3 with neutropenia at 680/mm 3 and thrombocytopenia at 12.000/mm 3 , the blood smear was without abnormality. These anomalies were associated with a hypoalbunemia, hypertriglyceridemia, hyperferritinemia, lactate dehydrogenase (LDH) level was at 337 IU/L, low prothrombin time (PT) at 56 % and fibrinogen level at 1 g/L. The direct Coombs test was positive. Examination of the myelogram revealed the presence of leishmania bodies and figures of hemophagocytosis. A diagnosis of visceral leishmaniasis associated with MAS was made. The patient was put on liposomal amphotericin B and corticosteroid therapy with good clinical and biological evolution and good therapeutic tolerance. Conclusion The association of VL and MAS remains rare and should be evoked even in non-endemic areas since late diagnosis worsens the prognosis and may even be responsible for the death of patients despite an aggressive treatment.

No abstract

Objective Coronavirus disease 2019 (COVID-19) is a viral disease caused by severe acute respiratory syndrome coronavirus 2. The clinical manifestations and the evolution of patients with COVID-19 are variable. In addition to respiratory involvement, COVID-19 leads to systemic involvement and can affect the hematopoietic system. This study aimed to evaluate the prognostic value of hematological and hemocytometric parameters in predicting the severity of patients with COVID-19. Methods We performed a retrospective study at Mohammed VI university Hospital from 1 March to 11 November 2020. We collected demographic characteristics and hematological findings of incident COVID-19 cases. Results A total of 245 patients were included in our study. We found that the rate of lymphopenia was significantly reduced in patients who were severely affected by COVID-19. Additionally, the rate of neutrophilia, the neutrophil side fluorescence light signal, monocyte fluorescent intensity, monocyte size, the neutrophil-to-lymphocyte ratio, the platelet-to-lymphocyte ratio, and the lymphocyte-to-monocyte ratio were significantly elevated in patients who were severely affected by COVID-19. Conclusions These results are consistent with the literature regarding the predictive value of these markers. A prospective validation in a large population with a longer follow-up is required.

The prevention of bleeding by prophylaxis regimen particularly for joint rehabilitation, could be considered a more effective treatment for hemophilia patients. In hemophiliacs with factor VIII inhibitor (F VIII inh) prophylaxis is not generally proposed because the bypassing agents for these patients may be less effective than F VIII concentrates. We report the regimen and results of a 6 months rFVIIa (Recombinant factor VIIa) prophylaxis, in a young hemophilia A patient (4 years old), with F VIII inh and immune tolerance induction (ITI) treatment and compared, with rFVIIa, the on demand treatment results for the 6 months prior to prophylaxis. After 2 years of a high dose regimen ITI, the FVIII inh titer was less than 50 BU and the immune tolerance treatment remains on going. Due to the development of a right knee target joint the rFVIIa prophylaxis was decided as an active rehabilitation approach to prevent the development of chronic arthropathy as well as to improve the quality of life of the child. During the 6 months period, prior to the initiation of rFVIIa prophylaxis 22 bleeds occurred i.e., 9 right knee hemarthrosis and 13 other joint bleedings and hematoma including elbow, wrist, ankle, foot, arm and chest. These bleeds were all treated with rFVIIa with a dose ranging from 100 to 200 μg/kg depending on the severity of the episodes and the duration of treatment ranged from 1 to 8 days. After 6 recurrent right knee hemarthrosis, a lavage of the joint was performed and prophylaxis with rFVIIa was subsequently initiated. A 120 μg/kg rFVIIa injection was performed 3 times a week concomitantly with the ITI treatment infusion and just before the physiotherapy course. During the 6 months of prophylaxis regimen we observed 9 bleeds with 3 major post traumatic bleedings which were treated by one 200 μg/kg/day rFVIIa injection which was resolved in one to three days. This prophylaxis treatment was effective for the arthropathy evolution and permitted the patient to return to school on a regular basis compared to the previous year. The total dose of on demand rFVIIa treatment used before prophylaxis was 458 mg/6 months. This amount decreased by 25% during the six months of prophylaxis with rFVIIa to reach 343 mg. The results of this significant observation led us to conclude that rFVIIa could be effectively used as prophylactic treatment in patients with FVIII inh and administered safely via a portacath device even in cases of high doses, as demonstrated in this young patient. This prevention approach resulted in a decrease of bleeding episodes, injections, and a significant improvement in the quality of life.