José Francisco Comenalli Marques

A randomised, prospective comparison of allogeneic bone marrow and peripheral blood progenitor cell transplantation in the treatment of haematological malignancies

We present the results of a prospective, randomised study comparing PBPC and BM focusing on engraftment, acute and chronic GVHD and survival. Forty patients with haematological malignancies received HLA-identical sibling BM (group A) or PBPC (group B). Evaluable patients were 19 (A) and 18 (B). Median age was 35 (17–56) in A and 29.5 (9–51) in B. Conditioning was mainly Bu-Cy2; GVHD prophylaxis was CSA-MTX. PBPC were harvested after 5 days of G-CSF 10 μg/kg/day. Median days for an ANC >0.5  × 109/l was 18 (13–30) in A and 16 (11–25) in B (P = 0.10). Platelets >20 × 109/l occurred at +17 (10–40) in A and +12 (9–36) in B (P = 0.01). The probability of ⩾2 grade a-GVHD was 19% (A) and 27% (B) (P = 0.53). The probability of all grade c-GVHD was 70% with BM. In spite of the small number of patients in group B (PBPC), our data suggest the great majority of them will have c-GVHD (P = 0.08); extensive disease was present in 50 and 100%, respectively (P = 0.05). The estimates of overall survival for A and B at 1000 days are 51 and 47%, respectively (P = 0.67); DFS at 1000 days are 52 and 58%, respectively (P = 0.50). PBPC resulted in faster platelet engraftment. The incidence of acute and chronic GVHD was similar in both groups, but the severity of c-GVHD was higher with PBPC. No differences in survival and DFS have been observed to date.

Clinical and histomorphometric evaluation of extraction sockets treated with an autologous bone marrow graft.

PURPOSE The aim of this study was to evaluate the potential of an autologous bone marrow graft in preserving the alveolar ridges following tooth extraction. MATERIALS Thirteen patients requiring extractions of 30 upper anterior teeth were enrolled in this study. They were randomized into two groups: seven patients with 15 teeth to be extracted in the test group and six patients with 15 teeth to be extracted in the control group. Hematologists collected 5 ml of bone marrow from the iliac crest of the patients in the test group immediately before the extractions. Following tooth extraction and elevation of a buccal full-thickness flap, titanium screws were positioned throughout the buccal to the lingual plate and were used as reference points for measurement purposes. The sockets were grafted with an autologous bone marrow in the test sites and nothing was grafted in the control sites. After 6 months, the sites were re-opened and bone loss measurements for thickness and height were taken. Additionally, before implant placement, bone cores were harvested and prepared for histologic and histomorphometric evaluation. RESULTS The test group showed better results (P<0.05) in preserving alveolar ridges for thickness, with 1.14+/-0.87 mm (median 1) of bone loss, compared with the control group, which had 2.46+/-0.4 mm (median 2.5) of bone loss. The height of bone loss on the buccal plate was also greater in the control group than in the test group (P<0.05), 1.17+/-0.26 mm (median 1) and 0.62+0.51 (median 0.5), respectively. In five locations in the control group, expansion or bone grafting complementary procedures were required to install implants while these procedures were not required for any of the locations in the test group. The histomorphometric analysis showed similar amounts of mineralized bone in both the control and the test groups, 42.87+/-11.33% (median 43.75%) and 45.47+/-7.21% (median 45%), respectively. CONCLUSION These findings suggest that the autologous bone marrow graft can contribute to alveolar bone repair after tooth extraction.

Third‐trimester erythrocytapheresis in pregnant patients with sickle cell disease

Objective: To evaluate the effects of prophylactic transfusion by means of erythrocytapheresis at the beginning of the third trimester of pregnancy in women with sickle cell disease (SCD). Methods: A cohort of 14 pregnant women with SCD who received prophylactic erythrocytapheresis transfusions at the beginning of the third trimester was retrospectively compared with a cohort of 17 pregnant women who received simple prophylactic transfusions for no indication other than SCD severity. Results: Prophylactic erythrocytapheresis transfusions were associated with a lower risk of intrauterine growth restriction (OR, 0.11; 95% confidence interval, 0.01–1.00) and oligohydramnios (OR, 0.65; 95% confidence interval, 0.45–0.92) in pregnant women with SCD. Conclusion: These results suggest that erythrocytapheresis transfusions are beneficial in women with SCD who are in the third trimester of pregnancy. Given the decrease in transfusion risks, this therapy deserves further evaluation in future trials.

Plasmapheresis in the treatment of myasthenia gravis: retrospective study of 26 patients

We analyzed the experience of Unicamp Clinical Hospital with plasma exchange (PE) therapy in myasthenia gravis (MG). About 17.8 % of a totality of MG patients had PE performed: 26 cases, 19 women and seven men. The mean age-onset of MG was 28 years, extremes 11 and 69. Minimum deficit observed in the group was graded IIb (O & G) or IIIa (MGFA scale). One patient had prethymectomy PE. In seven the procedures were performed due to myasthenic crisis and in 18 patients due to severe myasthenic symptoms or exacerbation of previous motor deficit. Two patients were also submitted to chronic PE considering refractoriness to other treatments. Twenty-six patients had 44 cycles of PE and 171 sessions. The mean number of sessions was 3.9 (SD +/- 1.4) each cycle; median 5, extremes 2 and 6. The mean time by session was 106,5 minutes (SD +/- 35.2); median 100.5 (extremes of 55 and 215). The mean volume of plasma exchanged in each session was 2396 ml (SD +/- 561); median 2225 (extremes 1512 and 4500). Side effects occurred: reversible hypotension (seven cases), mild tremor or paresthesias (seven cases). Infection and mortality rates due to PE were zero. All patients had immediate benefit of each PE cycle and usually they also received prednisone or other immunosuppressors. Good acceptance of the procedure was observed in 80.7% of patients.

An algorithm based on peripheral CD34+ cells and hemoglobin concentration provides a better optimization of apheresis than the application of a fixed CD34 threshold

BACKGROUND: Optimization of peripheral blood stem cell (PBSC) collection for autologous bone marrow transplantation is necessary for a good standard of care and cost‐effectiveness. An algorithm was validated for prediction of the day of maximum peripheral CD34+ cell concentration after mobilization chemotherapy (DayCD34peak).

The effects of exchange transfusion for prevention of complications during pregnancy of sickle hemoglobin C disease patients.

Pregnancy represents a challenge for women with sickle cell disease (SCD), with higher rates of both maternal and fetal complications. The aim of this study was to evaluate the impact of prophylactic transfusion support administered specifically to pregnant women with sickle hemoglobin C disease.

Is there justification for universal leukoreduction

The Associacao Brasileira de Hematologia, Hemoterapia e Terapia Celular (ABHH), the institution that represents the Associacao Medica Brasileira (AMB) in respect to hematology, transfusion therapy and cell therapy in Brazil hereby states: Prospective randomized studies have not demonstrated any positive clinical impact with the universal use of blood components in which the initial number of leukocytes has been reduced (universal leukoreduction). For this reason, universal leukoreduction remains a technically controversial topic. The current consensus is that leukoreduction has defined indications in the prevention of three blood transfusion complications only: (i) non-hemolytic febrile reactions when the patient has had this reaction previously; (ii) platelet refractoriness caused by alloimmunization against leukocyte antigens and (iii) the transmission of cytomegalovirus

Haemostatic management of extreme challenges to haemostasis in acquired von Willebrand syndrome

Summary.  Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic condition for which very little information is available regarding the management of extreme challenges to Haemostasis. The AVWS is more common in the elderly, who are frequently exposed to invasive procedures and/or chemotherapy. Haematopoietic stem cell transplantation (HSCT) is a situation in which the haemostatic capacity is challenged by severe thrombocytopaenia, chemotherapy‐associated mucosal barrier breakdown and the need for invasive procedures. In our report, we present and discuss the haemostatic management of a patient with AVWS who was refractory to Von Willebrand factor concentrate replacement during the course of an autologous HSCT to treat multiple myeloma. Patients with AVWS are frequently exposed to high‐risk haemostatic challenges, and additional information about the haemostatic management of these situations is necessary.

Guillain-Barré syndrome in the elderly: clinical, electrophysiological, therapeutic and outcome features

UNLABELLED There are few papers devoted to geriatric Guillain-Barré (GBS) and many related issues remain unanswered. OBJECTIVE To describe clinical, electrophysiological and therapeutic features in this age. METHOD Clinico-epidemiological data and therapy of GBS patients older than 60 years were reviewed. Hughes scores were used to quantify neurological deficit and define outcome. RESULTS Among 18 patients (mean age 64.8 years), 9 had evident prodrome and 80% noticed initially sensory-motor deficit. Demyelinating GBS was found in 8 and axonal in 6 subjects. There was one Miller-Fisher and 3 unclassified cases. Plasmapheresis (PFX) was single therapy in 12 patients and intravenous immunoglobulin (IVIg) in 2. Disability scores just before therapy were similar in both groups, so as short and long term outcome. CONCLUSION Axonal GBS seems to be more frequent in the elderly and this may have prognostic implications. PFX and IVIg were suitable options, but complications were noticed with PFX. Prospective studies are needed to better understand and manage GBS in the elderly.

Rasmussen's encephalitis: the relevance of neuropsychological assessment in patient's treatment and follow up

Rasmussens encephalitis is characterized by refractory epilepsy, neurological deterioration and progressive atrophy of one cerebral hemisphere. The objective of this study is to describe the importance of neuropsychological evaluation in the treatment decision and follow-up of patients with Rasmusseńs encephalitis. Neuropsychological assessment was performed in two steps. Firstly, the clinical history was obtained and the Vineland adaptative behavior scale (VABS) was applied. After this first step, the patients with social maturity level equal or higher than the inferior limit underwent a battery of neuropsychological assessment. We evaluated three patients before any specific treatment was started, and six months after the intervention (surgery or plasmapheresis). Patient 1 underwent left hemispherectomy and had global improvement on second neuropsychological assessment. This suggests that the decision of performing surgery was adequate. Patients 2 and 3 underwent plasmapheresis. They did not present cognitive decline between both evaluations which suggest that our decision of postponing surgery was adequate as well. We conclude that neuropsychological assessment is important when evaluating patients with Rasmusseńs encephalitis. That is especially true for patients in whom disease progression is slow, and surgery timing has to be carefully planned.