12 research outputs found
Prolonged complete hematologic response in relapsed/refractory T-large granular lymphocyte leukemia after bendamustine treatment
T-large granular lymphocyte
leukemia (T-LGLL) is a chronic clonal proliferation
of effector memory cytotoxic CD3+CD57+CD56- T
cells and the current guidelines suggest
immunosuppressive therapy as first-line therapy, but
the treatment of refractory/relapsed patients is still
challenging due to the lack of prospective studies.
We describe a series of two refractory/relapsed
T-LGLL patients successfully treated with
bendamustine, a chemotherapeutic agent largely used
for B-cell neoplasms, but poorly investigated for the
treatment of T-cell diseases. Complete remission
(CR) was achieved in 3 and 6 months, respectively,
and maintained for at least 20 months. One patient
relapsed after a 20-month CR, but she was
responsive to bendamustine therapy again, obtaining
a further prolonged CR.
Bendamustine as single agent or in combination
could be a feasible therapeutic option in
refractory/relapsed T-LGLL, especially for elderly
patients because of its safety profile
Accelerated bone mass senescence after hematopoietic stem cell transplantation
Osteoporosis and avascular necrosis
(AVN) are long-lasting and debilitating complications of hematopoietic stem cell transplantation
(HSCT).
We describe the magnitude of bone loss, AVN
and impairment in osteogenic cell compartment following autologous (auto) and allogeneic (allo)
HSCT, through the retrospective bone damage revaluation of 100 (50 auto- and 50 allo-HSCT) longterm survivors up to 15 years after transplant. Current treatment options for the management of these
complications are also outlined.
We found that auto- and allo-HSCT recipients
show accelerated bone mineral loss and microarchitectural deterioration during the first years after
transplant. Bone mass density (BMD) at the lumbar
spine, but not at the femur neck, may improve in
some patients after HSCT, suggesting more prolonged bone damage in cortical bone. Phalangeal
BMD values remained low for even more years,
suggesting persistent bone micro-architectural alterations after transplant. The incidence of AVN was
higher in allo-HSCT recipients compared to autoHSCT recipients. Steroid treatment length, but not
its cumulative dose was associated with a higher incidence of bone loss. Allo-HSCT recipients affected
by chronic graft versus host disease seem to be at
greater risk of continuous bone loss and AVN development. Reduced BMD and higher incidence of
AVN was partly related to a reduced regenerating
capacity of the normal marrow osteogenic cell compartment.
Our results suggest that all patients after autoHSCT and allo-HSCT should be evaluated for their
bone status and treated with anti-resorptive therapy
as soonas abnormalities are detected
OPSI threat in hematological patients
Overwhelming post-splenectomy infection (OPSI) is a rare medical emergency, mainly caused by encapsulated bacteria, shortly progressing from a mild flu-like syndrome to a fulminant, potentially fatal, sepsis. The risk of OPSI is higher in children and in patients with underlying benign or malignant hematological disorders.
We retrospectively assessed OPSI magnitude in a high risk cohort of 162 adult splenectomized patients with malignant (19%) and non malignant (81%) hematological diseases, over a 25-year period: 59 of them splenectomized after immunization against encapsulated bacteria, and 103, splenectomized in the previous 12-year study, receiving only life-long oral penicillin prophylaxis. The influence of splenectomy on the immune system, as well as the incidence, diagnosis, risk factors, preventive measures and management of OPSI are also outlined.
OPSI occurred in 7 patients (4%) with a median age of 37 years at time interval from splenectomy ranging from 10 days to 12 years. All OPSIs occurred in non immunized patients, except one fatal Staphylococcus aureus-mediated OPSI in a patient adequately immunized before splenectomy.
Our analysis further provides evidence that OPSI is a lifelong risk and that current immune prophylaxis significantly decreases OPSI development.
Improvement in patients’ education about long-term risk of OPSI and increased physician awareness to face a potentially lethal medical emergency, according to the current surviving sepsis guidelines, represent mandatory strategies for preventing and managing OPSI appropriately
Efficacy and safety of splenectomy in adult autoimmune hemolytic anemia
Autoimmune hemolytic anemia (AIHA) is a rare hematologic disease, primarily affecting adults or children with immunodeficiency disease. First-line therapy consists of long course of steroids administration, with an early complete response rate (CRr) of 75-80%, but up to 20-30% of patients requires a second-line therapy. Rituximab is the first choice in refractory old AIHA patients, because of its safety and efficacy (early CRr at 80-90% and at 68% at 2-3 years). For this reason, splenectomy is even less chosen as second-line therapy in elderly, even though laparoscopic technique decreased complication and mortality rates. However, splenectomy can be still considered a good therapeutic option with a CRr of 81% at 35.6 months in patients older than 60 year-old, when rituximab administration cannot be performed
Reproductive issues in patients undergoing Hematopoietic Stem Cell Transplantation: an update
In 1963 George Mathé announced to the world that he had cured a patient of leukaemia by means of a bone-marrow transplant. Since than much progress has been made and nowadays Hematopoietic Stem Cell Transplantation (HSCT) is considered the most effective treatment of numerous severe haematological diseases. Gynaecological complications in HSCT women represent a serious concern for these patients, but often underestimated by clinicians in the view of Overall Survival. The main gynaecological complications of HSCT are represented by: premature ovarian failure (POF), thrombocytopenia-associated menorrhagia, genital symptoms or sexual problems in course of chronic GVHD (cGVHD), osteoporosis, secondary solid tumours due to immunosuppressive drugs to treat cGVHD and severity of cGVHD, and fertility and pregnancy issues. In particular fertility-related issues are always more relevant for patients, whose life expectation is constantly growing up after HSCT.Thus, taking care of a patient undergoing HSCT should primarily include gynaecological evaluation, even before conditioning regimen or chemotherapy for the underlying malignancy, as, in our opinion, it is of great importance to ensure a complete diagnostic work-up and intervention options to guarantee maximum reproductive health and a better quality of life in HSCT women.The present review aims at describing principal features of the aforementioned gynaecological complications of HSCT, and to define, on the basis of current international literature, a specific protocol for the prevention, diagnosis, management and follow-up of gynaecological complications of both autologous and heterologous transplantation, before and after the procedure
Role of Laparoscopic Splenectomy in Elderly Immune Thrombocytopenia
The management of older patients with chronic primary immune thrombocytopenia (ITP) is still very challenging because of the fragility of older patients who frequently have severe comorbidities and/or disabilities. Corticosteroid-based first-line therapies fail in most of the cases and patients require a second-line treatment, choosing between rituximab, thrombopoietin-receptor agonists and splenectomy. The choice of the best treatment in elderly patients is a compromise between effectiveness and safety and laparoscopic splenectomy may be a good option with a complete remission rate of 67% at 60 months. But relapse and complication rates remain higher than in younger splenectomized ITP patients because elderly patients undergo splenectomy with unfavorable conditions (age >60 year-old, presence of comorbidities, or multiple previous treatments) which negatively influence the outcome, regardless the hematological response. For these reasons, a good management of concomitant diseases and the option to not use the splenectomy as the last possible treatment could improve the outcome of old splenectomized patients
Low-dose valgancyclovir as cytomegalovirus reactivation prophylaxis in allogeneic hematopoietic stem cell transplantation
Successful management of pulmonary mucormycosis with liposomal amphotericin B and surgery treatment: a case report
Mesenchymal Stem Cells from the Wharton’s Jelly of the Human Umbilical Cord: Biological Properties and Therapeutic Potential
Accelerated and persistent bone loss after autologous and allogeneic stem cell transplantation
Osteoporosis and avascular necrosis (AVN) are long-lasting and debilitating
complications of hematopoietic stem cell transplantation (HSCT).
We describe the magnitude of bone loss, AVN and impairment in
osteogenic cell compartment following autologous (auto) and allogeneic
(allo) HSCT, through the retrospective bone damage revaluation of 100
(50 auto- and 50 allo-HSCT) long-term survivors up to 15 years after
HSCT. Age at transplantation of HSCT recipients ranged between 18 and
50 years (median, 30) and their post-HSCT follow-up lasted from 1 to
15 years (median, 6). Primary diseases were acute (n=44) or chronic
(n=13) myeloid leukemia, Hodgkin disease (n=17), non-Hodgkin lymphoma
(n=12), and multiple myeloma (n=14). Bone mass density (BMD)
was measured by dual energy X-ray absorptiometry (DEXA) at lumbar
spine (LS, L1-L4) and femoral neck (FN), and by quantitative phalangeal
ultrasonometry. At the time of testing (mean follow-up after HSCT: 65
months; range: 1-15 years), LS (Z score mean: -0.4 and -0.9 in auto- and
in allo-HSCT recipients, respectively; p<0.05), FN (-0.6 and -1.4 in autoand
in allo-HSCT recipients, respectively; p<0.05) and phalanges (-1 and
-1.5 in auto- and in allo-HSCT recipients, respectively; p<0.05) BMD
were significantly reduced in comparison with BMD of 100 healthy controls
(p<0.001 in all examined sites), suggesting more prolonged bone
damage in cortical than in trabecular bone. BMD at LS, but not at FN,
improved 3 years after HSCT in some patients, suggesting more prolonged
bone damage in cortical than in trabecular bone. Phalangeal BMD
values remained low for even more years, suggesting persistent bone
micro-architectural alterations after HSCT. Eight patients developed
AVN, 1 to 15 years following HSCT: 6 (12%) after allo-HSCT and 2 (4%)
after auto-HSCT (p<0.05). Development of acute and chronic graft versus-
host disease, was associated with both a more severe BMD reduction
in all bone sites (<0.05) and with higher frequence of AVN. Reduced
BMD and higher incidence of AVN was partly related to a reduced regenerating
capacity of the normal marrow osteogenic cell compartment,
measured by growing colony-forming units-osteogenic cells. Our results
document that an accelerated bone mineral loss and micro-architectural
deterioration occurs during the first years after HSCT and is more
severe in the allogeneic setting, suggesting that all patients early after
auto-HSCT and allo-HSCT should be evaluated for their bone statu