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PubMed1992

[Acid-base disturbances in hematologic diseases].

In this article, the acid-base disturbances encountered in hematologic diseases are discussed. Occurrence of lactic acidosis (LA) without obvious clinical tissue hypoxia has been reported in patients with leukemia and lymphoma. Most of the patients with LA had liver involvement and clinical evidence of impaired hepatic function, suggesting that both increased production and decreased lactate metabolism are necessary for the development of LA in leukemia and lymphoma. Acute tumor lysis syndrome consists of hyperuricemia, hyperpotassemia, and hyperphosphatemia with hypocalcemia following neoplastic cell lysis, particularly in lymphoproliferative disorders. In patients with multiple myeloma (MM), proximal renal tubular acidosis (Fanconi syndrome) associated with Bence Jones proteinuria has been reported. In addition, MM is one of the first conditions recognized to be associated with lower anion gap.

Acidosis, Lactic↗

PubMed2001

Allogeneic peripheral blood stem cell transplantation (PBSCT) from HLA-identical sibling donors in children with hematological diseases: a single center pilot study.

Between February 1995 and July 1999 25 pediatric patients (8 months to 14 years old) underwent peripheral blood stem cell transplantation (PBSCT) from an HLA-identical sibling donor. Diagnoses included ALL (17), non-ALL (6), and non-malignant disease (2). GVHD prophylaxis consisted of cyclosporine plus methotrexate (15), only cyclosporine (8), cyclosporine plus prednisone (1), or nothing (1). All donors (6 months to 41 years old) received G-CSF at 10 microg/kg/day subcutaneously for 4-5 days and on day 5 underwent large volume leukapheresis. Median number of CD34(+) and CD3(+) cells collected and infused was 6.9 x 10(6) (range 2.5-32.8) and 4.5 x 10(8) (0.5-22.1) per kg of recipient body weight respectively. Median time to achieve ANC >0.5 x 10(9)/l and platelets >20 x 10(9)/l was 10 and 12 days, respectively. Acute GVHD grade > or =II developed in 10 of 24 evaluable patients (42%). Probability of acute GVHD was 62%. Median time to discharge was 25 days (range 14-52). Among 20 evaluable patients, five (25%) developed chronic GVHD at day 100. Probability of chronic GVHD was 29% after 1 year post PBSC. At a median follow-up of 558 (9-2071) days, overall survival for the whole group is 68%. Probabilities of event-free survival, overall survival and relapse for patients with malignant hematological diseases are 53%, 59% and 24% at 5 years, respectively. This study has confirmed the feasibility and safety of mobilization and collection of PBSC products and the applicability of this procedure to the pediatric population, both donors and recipients. Studies including larger numbers of pediatric patients undergoing allogeneic PBSCT are warranted to determine the long-term outcomes of such procedures.

Adolescent↗

PubMed2003

[Treatment of malignant hematologic diseases by peripheral blood stem cell transplantation combined with halotype lymphocyte infusion].

In order to observe the curative and side effects in malignant hematologic diseases treated with autologous peripheral blood stem cell transplantation (auto-PBSCT) combined with halotype lymphocyte infusion, auto-PBSCs were mobilized, harvested and stored at -196 degrees C from patients in first CR or PR with intensive chemotherapy (Ara-C 1.0 g/m(2) x 5 days or cyclophosphamide 60 mg/kg x 2 days) and G-CSF. Unpurged auto-PBSCs were infused when patients received the conditioning regimen with busulfan, total irradiation or cyclophosphamide. Halotype lymphocytes [mean 5.0 x 10(7)/kg, (4.5 - 6.5) x 10(7)/kg] irradiated with 7.5 Gy were infused to patients when WBCs were more than 1 x 10(9)/L. Hematopoietic recovery and survival of patients were observed. The results showed that in 12 cases accepted this protocol, five patients with acute non-lymphocytic leukemia got to durable remission, of which 2 had durable remission of more than 50 months. One of three patients with non-Hodgkin's lymphoma IVb reached durable remission, and two relapsed and died on 4 and 6 months after treatment, respectively. Two CML patients were also achieved durable remission. One patient with multiple myeloma relapsed on 36 months later, but he still survived disease-free with treatment of thalidomide. In a follow-up survey of 25 months, the disease-free survival was 83%. No severe side effects were observed except platelet delayed recovery after halotype lymphocyte infusion. STR-PCR analysis showed that infused donor lymphocytes disappeared in 3 recipients at 72 hours after infusion. It is concluded that auto-PBSCT combined with halotype lymphocyte infusion could decrease the relapse of malignant hematologic diseases and improve the effect of auto-PBSCT. Recovery of platelet, however, could be delayed by halotype lymphocyte infusion.

Adult↗

PubMed1997

Should repeated partial splenectomy be attempted in patients with hematological diseases? Technical pitfalls and causes of failure in Gaucher's disease.

BACKGROUND/PURPOSE: The awareness of the risk of overwhelming sepsis after splenectomy prompted surgeons to attempt splenic preservation in patients who had hematologic diseases for which splenectomy was the conventional treatment. Partial splenectomy for Gaucher's disease was widely performed before the introduction of alglucerase. In sporadic cases a second partial splenectomy had also been attempted. METHODS: The authors present three cases of failed repeated partial splenectomy attempted before alglucerase was available. The role of angiography in planning operative strategy and the surgical pitfalls of this unusual reintervention are discussed. CONCLUSION: New indications for partial splenectomy in other hematologic diseases makes the experience gained with Gaucher's disease valuable for management decisions.

Adolescent↗

PubMed2005

[Pulmonary fungal infection in malignant hematological diseases: an analysis of 14 cases].

The objective of this study was to evaluate the etiological factors, diagnosis and therapy of pulmonary fungal infection in hematological malignancies, 14 cases of malignant hematological disease with pulmonary fungal infection were collected and analyzed. The results showed that 11 out of 14 cases had the respiratory manifestations, sputum was sticky and difficult to be expectorated in 7 cases, X rays in chests showed shadows with features of stigma and sheet in 11 cases, Candida albicans and aspergillus infection were observed in 10 and 2 cases respectively, the numbers of neutrophil were below 0.5 x 10(9)/L in 8 cases and below 1.0 x 10(9)/L in 3 cases respectively, fluconazole was used for 12 cases and clinical symptoms of 11 cases were improved within two weeks. In conclusion, the occurrence of pulmonary fungal infection in malignant hematological diseases is associated with intensive chemotherapy, decrease of neutrophil counts and using of broad-spectrum antibiotics, the diagnosis at early stage is difficult and clinicians should pay more attention to its clinical and laboratory examinations, and give them therapy in time.

Adolescent↗

PubMed2004

[Study on bone marrow microvascular density in hematological diseases].

To investigate the state and significance of bone marrow angiogenesis in hematological diseases, bone marrow microvascular density (BM-MVD) in plastic-embedded section was examined using acetone-fixed bone marrow tissues embedded in glycol-methacrylate (GMA) resin and by the method of immunohistochemistry. The results showed that bone marrow MVD increased greatly in newly diagnosed hematological malignancies before treatment. BM-MVD in patients with acute leukemia decreased down to the normal range as the controls at the time of complete remission. In the non-remission group, BM-MVD decreased less, but when relapsed it increased again up to the same range as the newly diagnosed hematological malignancies, significant increase of BM-MVD was found in patients with anemia, but in less degree than that in hematological malignancies. It is concluded that bone marrow angiogenesis plays a key role in the pathogenesis and development of hematological malignancy. Antiangiogenic therapy may be able to constitute a novel strategy for the treatment of hematological malignancies including leukemia.

Acute Disease↗

PubMed2001

[Nonmyeloablative Allogeneic Peripheral Blood Hematopoietic Stem Cell Transplantation for Therapy of Hematologic Diseases]

The objectve of this investigation was to explore the use of nonmyeloablative allogeneic peripheral blood stem cell tranplantation for treatment of hematologic diseases. Six patients were included: 3 cases with acute leukemia in first complete remission (2 AML and 1 ALL), 2 severe aplastic anemias and 1 chronic myeloid leukemia in chronic phase. All of the 6 cases were received HLA-identical siblings donor peripheral blood stem cell transplantation after a nonmyeloablative conditioning. The donor cells were engrafted in all patients (3 cases were full engrafment of donor cells and 3 were mixed chimerism). Hematopoietic recovery was appeared in all of the cases (ANC recovered to more than 0.5 x 10(9)/L and platelet count to more than 30 x 10(9)/L on day 9 to day 21 and day 14 to day 28 after transplantation, respectively). Two patients developed III or IV degree GVHD. Our preliminary results suggest that the procedure is more safe, efficient and less complications than myeloablative conditioning regimens and represents another new approach in the management of patients with hematologic diseases.

Journal Article↗

PubMedDate not supplied

[Recurrent infections in hematologic diseases treated with cefoxitine (author's transl)].

Forty febrile patients during several hematologic diseases (28 acute leukemias, 8 lymphocytic chronic leukemias, 3 drug induced agranulocytosis, 1 myeloid aplasia) received intensive combination antibiotherapy including cefoxitin (with gentamicin in all cases : Carbenicillin was added in 17 cases). Thirty-five patients had severe neutropenia before treatment. Infection's regression was obtained in 35 cases (in spite of persistence of neutropenia in 20 cases). No local, general or biological toxicity was observed.

Adolescent↗

PubMed1997

[Study on serum soluble interleukin-6 receptor levels in patients with malignant hematological diseases].

OBJECTIVE: To explore the clinical implication of soluble interleukin-6 receptor(sIL-6R) in patients with malignant hematological diseases. METHODS: Serum levels of sIL-6R in 26 multiple myeloma (MM), 34 acute leukemia (AL) and 17 non-Hodgkin's lymphoma (NHL) patients before and after chemotherapy and 20 normal controls were measured by immunoenzymetic assay. RESULTS: Serum levels of sIL-6R in MM and B-ALL patients at diagnosis were significantly higher than that in normal controls (P < 0.001, and P < 0.01, respectively), while the levels in ANLL, T-ALL and NHL patients were normal. Serum beta2 microglobulin and creatinine levels in MM patients with elevated serum sIL-6R were higher than that in patients with normal sIL-6R levels(P < 0.001, and P < 0.05, respectively). Serum sIL-6R levels did not relate to clinical stages. Serum lactic-dehydrogenase (LDH) levels in B-ALL patients with elevated sIL-6R were higher than that in patients with normal sIL-6R levels (P < 0.05). Serial measurement of serum sIL-6R in MM patients showed a good correlation with serum M-protein and plasma cell percentage in bone marrow. CONCLUSION: Serum sIL-6R levels were significantly increased in MM and B-ALL patients, and it is a predictive factor for responsiveness to chemotherapy and an indicator of disease activity.

Adult↗

PubMedDate not supplied

Reactivity of platelets from healthy individuals and from patients with hematologic diseases following incubation with pegylated recombinant human megakaryocyte growth and development factor in vitro.

Platelets express the receptor for thrombopoietin. It is possible that thrombopoietin modulates platelet reactivity. We examined the effect of pegylated recombinant human megakaryocyte growth and development factor (PEG-rHuMGDF) on platelet activation in vitro using flow cytometry. We compared samples from healthy individuals and from patients with various hematologic diseases (AML, myeloma, postchemotherapy). Citrated whole blood was incubated with PEG-rHuMGDF (10 or 100 ng/ml), then a mild stimulus was added (0.1 U thrombin/ml). Blood from healthy individuals showed a significantly higher degree of platelet activation (CD62p expression), microparticle generation, and aggregate formation after incubation with PEG-rHuMGDF+thrombin versus thrombin alone (p < 0.05). However, this difference could not be shown for platelets from patients with thrombocytopenia or other hematologic diseases. The use of PEG-rHuMGDF should be safe and not cause an additional risk of thromboocclusive disease in these patients.

Adult↗

PubMed2006

GATA transcription factors and hematological diseases.

The development of mature blood cells from hematopoietic stem cells is regulated by transcription factors that control and coordinate the expression of lineage-specific genes. The GATA family consists of six transcription factors that function in hematopoietic and endodermal development. Among them, GATA-1 is expressed in erythroid, megakaryocytic, eosinophil and mast cell lineages, and GATA-2 is expressed in stem and progenitor cells, at more immature stage compared with GATA-1. Based on the characteristic phenotypes of GATA-1 and GATA-2 mutant mice, it has been suggested that mutations of these GATA genes in humans may result in the onset of certain clinical diseases. To date, mutations of GATA-1 gene have been found in inherited anemia and thrombocytopenia, and Down syndrome-related acute leukemia, which exhibits megakaryocytic phenotypes and frequently occurs in patients with Down syndrome. In contrast, no mutation of GATA-2 gene has been identified in hematological diseases; however, we found the expression level of GATA-2 is significantly decreased in CD34 positive cells in patients with aplastic anemia. Since GATA-2 functions in the proliferation of hematopoietic stem cells, the reduction of GATA-2 expression in CD34 positive cells may result in the decreased number of hematopoietic stem cells, which is the characteristic feature of aplastic anemia. Based on these lines of evidence, some types of hematological diseases may be defined as transcription factor diseases.

Anemia, Aplastic↗

PubMed1999

Laparoscopic vs open splenectomy in the management of hematologic diseases.

BACKGROUND: Laparoscopic splenectomy (LS) is becoming the gold standard in the treatment of several splenic diseases. Shorter postoperative stay and more rapid return to full activity are the primary advantages of LS. METHODS: Prospective data collection of 44 consecutive LS (group 1) and comparison with a historical control group of 56 consecutive open splenectomies (OS) (group 2) were performed for hematologic diseases. RESULTS: The LS patients started earlier on an oral diet (p < 0.0001) and left the hospital sooner (p < 0.0002) than OS patients. Less blood transfusion (p < 0.004) and pain medication (p < 0.0001) was required by LS patients. They also had fewer postoperative complications (p < 0.03). Compared by diagnosis, patients with laparoscopic idiopathic thrombocytopenic purpura or Hodgkin's disease started to eat earlier (p < 0.0001) and left the hospital sooner (p < 0.01). Multivariate analysis showed that time to oral diet and postoperative stay was related to operative technique and age. Morbidity and pain medications were related, respectively, to transfusion requirements and type of surgical approach. CONCLUSIONS: Used to manage hematologic diseases, LS is feasible, effective, and safe. It offers several advantages over the open approach. The type of surgical approach seems to be the crucial factor in determining the length of the postoperative course.

Adolescent↗

PubMed1998

Inadequate detection of accessory spleens and splenosis with laparoscopic splenectomy. A shortcoming of the laparoscopic approach in hematologic diseases.

BACKGROUND: The ultimate goal of surgery for hematological disorders is the complete removal of both the spleen and accessory spleens in order to avoid recurrence of the disease. Whereas splenectomy by open surgery provides excellent results, the validity of laparoscopic splenectomy in this regard remains unknown. OBJECTIVE: The purpose of this study was to evaluate the detection of accessory spleens during laparoscopic splenectomy for hematologic diseases. METHODS: We therefore evaluated the pre-, intra-, and postoperative detection of accessory spleens in a consecutive series of 18 patients treated by elective laparoscopic splenectomy for hematological diseases by using computed tomography (CT) and denatured red blood cell scintigraphy (DRBCS). RESULTS: Preoperative CT, DRBCS, and laparoscopic exploration detected 25%, 25%, and 75% of accessory spleens, respectively. At time of laparoscopy, 16 accessory spleens were detected in seven of the 18 patients (41%). In two patients (11%), laparoscopic exploration failed to detect accessory spleens, whereas preoperative CT (one case) and DRBCS (one case) did reveal them. Postoperatively, during a mean follow-up of 28 months (median, 24; range, 12-44 months), nine patients (50%) showed persistence of splenic tissue by DRBCS, and three of them had signs of disease recurrence. CONCLUSIONS: This prospective clinical study suggests that elective laparoscopic surgery for hematological diseases does not allow complete detection of accessory spleens. Moreover, after such a laparoscopic approach, residual splenic tissue is detectable in half of the patients during the follow-up.

Adolescent↗

PubMed1995

[Allogenic bone marrow transplantation in the treatment of malignant hematologic disease].

We have treated 28 patients (pts) with malignant hematological diseases with allogeneic bone marrow transplantation (BMT). 18 pts had acute lymphoblastic (ALL) and non lymphoblastic leukemia (ANLL), 5 chronic myeloid leukemia (CML), 2 severe aplastic anemia (SAA), 1 myelodisplasia, 1 Fanconi's anemia and 1 advanced Non Hodgkin's lymphoma. All but three received the graft from HLA identical sibling donors. We used conditioning with total body irradiation and chemotherapy (cyclophosphamide, cytarabine and etoposide) in 17 pts and chemotherapy alone in 11. 24 pts had full hematological recovery 18 to 25 days post BMT. 15 pts died after BMT as a consequence of toxicity or early infection (4), graft failure (2), graft versus host disease (4) or relapse (5). Actuarial event free survival for the group with favorable prognosis (SAA, ALL and ANLL in first or second remission and CML in chronic phase) is 57% at 36 months. Allogeneic BMT is an effective and feasible therapeutic procedure for selected patients with hematological malignancies.

Actuarial Analysis↗

PubMed2004

Improved outcome of zygomycosis in patients with hematological diseases?

Zygomycosis is an opportunistic fungal infection that is increasingly reported in hematological patients. We describe 2 cases of successfully treated rhino-cerebral zygomycosis and give an overview of 120 patients from the literature with underlying hematological or oncological disorders. These data document the improved survival in sinus (15/17 patients surviving) and cutaneous (6/9 patients surviving) disease. Hematological patients with pulmonary (9/30 patients surviving) or disseminated (4/38 patients surviving) zygomycosis still have a poor prognosis. The clinical course of sinus-orbital involvement (4/11 patients surviving) follows sinus-cerebral (2/3 patients surviving) or cerebral (3/6 patients surviving) disease. Besides deoxycholate amphotericin B (AmB) (24/62 patients surviving), patients seem to benefit from liposomal amphotericin B (L-AmB) (10/16 patients surviving) or sequential AmB/L-AmB treatment (6/8 patients surviving). Alternative treatment options lead only in a few patients to success.

Adult↗

PubMed1984

Splenectomy for hematologic disease. The UCLA experience with 306 patients.

Between 1956 and 1981, 306 splenectomies for hematologic diseases were performed at the UCLA Medical Center. Of these operations, more than 75% were performed for therapeutic reasons to control anemia, thrombocytopenia, neutropenia, or painful symptoms of splenomegaly. Of the 65 patients who had idiopathic thrombocytopenic purpura, 77% showed an excellent response, and of the 39 patients who had hereditary spherocytosis, 90% responded. Other diseases with predictably good response rates were autoimmune hemolytic anemias, Felty's syndrome, and hairy cell leukemia. Forty patients with Hodgkin's disease had splenectomies for diagnostic purposes the last 10 years. The overall morbidity and mortality were 24% and 6%, respectively, the most common complications being pneumonia, wound infections, and local postoperative bleeding, and the most common cause of death being sepsis. The review supports the thesis that in carefully selected patients, therapeutic splenectomy can have desirable palliative effects and that diagnostic splenectomy has a sufficiently low risk to warrant its consideration in patients with Hodgkin's disease.

Adolescent↗

PubMed1999

Human herpesvirus 8 in hematologic diseases.

Human herpesvirus type 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV) is a new member of the g-herpesvirus family. It is an unusual herpesvirus in that it carries a large number of genes that encode oncoproteins or cell signaling proteins. In addition to being the causative agent of both HIV-associated and non-HIV-associated Kaposi's sarcoma this DNA tumor virus has been implicated in the pathogenesis of several diseases. These include multiple myeloma (MM), Waldenstöm's macroglobulinemia (WM), multicentric Castleman's disease (MCD), body cavity-based lymphoma (BCBL), and various other conditions such as sarcoidosis and pemphigus. While the causative role of the viral infection is fairly certain in the development of BCBL and multicentric Castleman's disease, HHV-8 may act through a different mechanism to induce plasma cell malignancies. It has been suggested though the finding is still controversial - that infection of bone marrow stromal dendritic cells by HHV-8 might be a key factor in the etiology and pathogenesis of monoclonal gammopathies. The aim of this review is to provide a short introduction into the tumorigenic potential of HHV-8 as well as to detail the available data and possible mechanisms on the involvement of this virus in different hematologic diseases.

Bone Marrow↗

PubMedDate not supplied

Autoimmune hemolytic anemia during alpha interferon treatment in nine patients with hematological diseases.

BACKGROUND. A number of side effects have been observed in patients treated for hematological diseases with alpha-interferon (IFN). In several cases side effects consisted of immunological disorders. Autoimmune hemolytic anemia (AIHA) is the most typical example of an IFN-induced immune-mediated complication. CASE SERIES. In 10 years we observed 9 patients with various hematological disorders who developed AIHA during IFN treatment. The interval between the start of IFN treatment and the onset of acute hemolysis suggests a dual pattern of occurrence: (1) early onset (interval 1 to 21 days), seen in patients who had anti-RBC antibodies before IFN treatment; (2) late onset (interval 3-38 months), in patients with no history of anti-RBC antibodies at the start of treatment. Discontinuation of IFN, often associated with prednisone treatment, caused prompt hematological recovery in all cases; anti-erythrocyte antibodies persisted in the first group of patients and disappeared in the second. CONCLUSIONS. In rare cases IFN may cause AIHA. The immunological derangement caused by IFN seems to act at two different levels: enhanced destruction of antibody-coated RBCs and induction of autoreactive B-cells. As for the possibility of other preexisting immunological disorders, AIHA (even latent) is a contraindication to IFN treatment. Patients treated with IFN need accurate monitoring to guard against for the development of autoimmune disorders.

Aged↗