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PubMed2004

[Conventional and new laboratory parameters in the evaluation of hematologic disease].

Laboratory investigation plays a crucial role in the workup of hematological disease. The well established method of morphological analysis of blood components has been continuously complemented by other methods. On one hand, these consist of considerable improvements of methods employed for automated cell enumeration allowing for early and accurate detection of cell subpopulations, and quantification of valuable red cell parameters, which are of use in the differential diagnosis of anemia. On the other hand, several parameters for the differentiation of microcytic anemia have become available often allowing for the sometimes difficult diagnosis of anemia of chronic disease, iron deficiency anemia, or thalassemia (ferritin, soluble transferrin receptor, transferrin saturation, RDW, zinc protoporphyrin, as well as reticulocyte indices CHr, Ret-Y Hypo%). In macrocytic anemia, introduction of methods to measure methylmalonic acid (MMA), homocystein, holotranscobalamin (holo-TC), complement the determinations of vitamin concentrations (vitamin B12, folic acid in serum and erythrocytes). Employing these newer parameters in addition to the well established ones allows for detection of early or combined disease. The clinician has to know the diagnostic characteristics not only of the old but also of the newer parameters.

Anemia, Iron-Deficiency↗
PubMed1988

Fatal legionellosis in patients with malignant hematologic diseases.

Pneumonia was present in 70/157 (44.6%) autopsied patients with malignant hematologic diseases. In 16/70 patients (22.9%), legionellae were found to be the causative agents by screening lung tissue specimens with the direct fluorescent antibody method. In 5/16 patients with Legionella pneumonia, in whom legionellosis had been suspected clinically, the diagnosis had already been established by serology, urinary Legionella antigen detection, and culture. These results provide evidence that legionellosis is an important pneumonia etiology in patients with malignant hematological diseases. Thus, Legionella diagnostics should be applied routinely, and antibiotics effective in the treatment of legionellosis should be added to the usual therapy in patients with etiologically unexplained pneumonias. In view of the common occurrence of relapses of Legionella pneumonia, antibiotic therapy should be continued for an extended period.

Aged↗
PubMed1996

Risks of synchronous gastrointestinal or biliary surgery with splenectomy for hematologic disease.

BACKGROUND: The addition of splenectomy to a gastrointestinal (GI) operation may have an adverse effect on mortality, morbidity, and even survival. OBJECTIVE: To determine the risks of the converse: synchronous GI surgery appended to splenectomy for hematologic diseases. DESIGN: Retrospective cohort. SETTING: Multiple hospitals comprising an affiliated surgical training program. PATIENTS: Consecutive sample of 207 adults (mean age, 49 years) with splenectomies for hematologic diseases. INTERVENTION: Splenectomy and concomitant GI or biliary surgery (group 1, n=19) and splenectomy alone (group 2, n=188). MAIN OUTCOME MEASURES: Length of hospital or intensive care unit stay, later operations, postoperative infections, postoperative abdominal abscess, major complications, and death. RESULTS: Preoperative and intraoperative factors were similar in both groups. Operative mortality was 3 of 19 in group 1 and 8 of 188 in group 2 (p=.07). The mean number of major complications tended to be higher in group 1 (1.5 vs 0.5, P=07). Despite no difference between the incidences of overall postoperative infections, patients in group 1 were much more likely to develop an abdominal abscess (4 of 19 vs 3 of 188, P=.002). Logistic regression established that patients undergoing splenectomy and synchronous GI or biliary surgery were 25 times more likely to develop an intra-abdominal abscess than were patients with splenectomy alone, even controlling for confounding factors (odds ratio, 24.7; 95% confidence interval, 3.1 to 196; P=.002). CONCLUSIONS: Synchronous GI or biliary surgery with splenectomy for hematologic disease increases the risk of intra-abdominal abscess and should be avoided. Complication and mortality rates may also be increased.

Abdominal Abscess↗
PubMed1978

Peliosis hepatis in hematologic disease. Report of two cases.

Two patients with hematologic disease, one of whom had received androgenic steroids, had liver damage associated with peliosis hepatis. In one patient with spherocytic hemolytic anemia, peliosis hepatis was an incidental postmortem finding. In the other patient, who was treated with androgenic-anabolic steroids for aplastic anemia, hepatic failure associated with peliosis hepatis developed. Splenic involvement by peliosis was present in both patients. Peliosis hepatis should be considered in the differential diagnosis of hepatic disease in patients with hematologic disorders, especially if treatment has included androgenic-anabolic steroids.

Anemia, Aplastic↗
PubMed1995

Systemic lupus erythematosus: emerging concepts. Part 1: Renal, neuropsychiatric, cardiovascular, pulmonary, and hematologic disease.

PURPOSE: To review advances and controversies in the diagnosis and management of systemic lupus erythematosus with visceral involvement (renal, neuropsychiatric, cardiopulmonary, and hematologic disease). DATA SOURCES AND STUDY SELECTION: Review of the English-language medical literature with emphasis on articles published in the last 5 years. More than 400 articles were reviewed. DATA SYNTHESIS: Recent debates pertaining to lupus nephritis have focused on the value of kidney biopsy data and the role of cytotoxic drug therapies. Many studies have shown that estimates of prognosis are enhanced by consideration of clinical, demographic, and histologic features. For patients with severe lupus nephritis, an extended course of pulse cyclophosphamide therapy is more effective than a 6-month course of pulse methylprednisolone therapy in preserving renal function. Adding a quarterly maintenance regimen to monthly pulse cyclophosphamide therapy reduces the rate of exacerbations. Plasmapheresis appears not to enhance the effectiveness of prednisone and daily oral cyclophosphamide. Small case series have shown pulses of cyclophosphamide to be beneficial in patients with lupus and neuropsychiatric disease refractory to glucocorticoid therapy, acute pulmonary disease (pneumonitis or hemorrhage), and thrombocytopenia. Patients with systemic lupus erythematosus have an increased prevalence of valvular and atherosclerotic heart disease, apparently because of factors related to the disease itself and to drug therapy. CONCLUSIONS: Cytotoxic agents are superior to glucocorticoid therapy for the treatment of proliferative lupus nephritis, but the optimal duration and intensity of cytotoxic therapy remain undefined. Definitive studies of the treatment of autoimmune thrombocytopenia and acute pulmonary disease and of the diagnosis and treatment of neuropsychiatric disease are not available.

Cardiovascular Diseases↗
PubMed1982

Translocations involving chromosomes #3 and #12: hematologic diseases associated with abnormalities of these chromosomes.

Two hematologic cases with translocations involving chromosomes #3 and #12 are described. The first case is that of a myeloproliferative disorder (preleukemia?) associated with a (3;12)(q29;q24) translocation in the bone marrow cells. No evidence of leukemic transformation has appeared to date. The second case is that of acute leukemia (AL) (M4 type) in which leukemic cells with t(3;12)(p14;q24) were seen. The roles of chromosomes #3 and #12 in hematopoiesis are considered, and the abnormalities affecting these chromosomes in various hematologic disorders have been tabulated and correlated. Abnormalities in chromosomes #3 and #12 appear to be common and nonrandom in hematologic diseases of a premalignant and a malignant type.

Acute Disease↗
PubMed1992

[Clinical effects of cefodizime in severe infections complicated with hematological diseases. Hanshin Infection Study Group].

One hundred thirty-eight patients with severe infections accompanying hematological diseases were treated with cefodizime (CDZM). CDZM was administered by intravenous injection at daily doses ranging from 2 g to 8 g for about 3 to 18 days. Clinical efficacies of CDZM were excellent in 30 cases, good in 37 cases, fair in 1 case and poor in 53 cases. The efficacy rate was 55.4%. The efficacy rate was 56.4% in patients from whom causative organisms were not identified. The efficacy rate against Gram-negative bacteria (66.7%) was higher than that against Gram-positive bacteria (28.6%). No significant differences in the efficacy rates were found among groups of patients with different initial neutrophil counts (less than 100, 100-499, and over 500/microliters). Skin eruption caused by CDZM was observed in 2 patients (1.5%). Hepatic disorders, renal dysfunction and urinary test abnormality (protein and urobilinogen) were observed in 7 (5.0%), 1 (0.7%) and 1 (0.7%), respectively. Therefore, CDZM was considered to be a clinically useful antibiotic for severe infections complicated with hematological diseases.

Adolescent↗
PubMed2004

Thromboembolic complications after splenectomy for hematologic diseases.

Thromboembolic complications following splenectomy for hematologic diseases occur in up to 10% of patients and may range from portal vein thrombosis (PVT) to pulmonary embolism (PE) and deep vein thrombosis (DVT). Up to now there exist no recommendations for the duration and intensity of prophylactic anticoagulation, which usually follows local institutional protocols. We report on three consecutive patients with severe portal vein thrombosis and/or pulmonary embolism--one with fatal outcome--7 to 35 days after splenectomy for autoimmune hemolytic anemia, immunothrombocytopenia, and indolent lymphoma, respectively. Incidence and pathophysiology of thromboembolic events (TE) in this patient group as well as prophylactic anticoagulation will be discussed, including a review of the current literature on this topic.

Adult↗
PubMed1995

[Object analysis of bone marrow MR imaging using double echo STIR sequence in hematological diseases].

The bone marrow of 84 patients with hematological disorders was investigated using short inversion time inversion recovery sequence (STIR) on an 1.5 Tesla superconducting MRI system. Double echo times of 20 and 100msec were applied to research the signal characteristics of the lesion and carry out quantitative analysis of the receiver operating characteristic curve (ROC). The hematological diseases included 19 cases of myelodysplastic syndrome (MDS), 18 of multiple myeloma (MM), 18 of chronic myelocytic leukemia (CML), 9 of aplastic anemia (AA), 8 of acute myelocytic leukemia (AML), 3 of chronic lymphocytic leukemia (CLL), 3 of myelofibrosis, and 3 others. Using STIR with double echo times, bone marrow showed high signal intensity (SI) on short TE and low SI on long TE in MDS and CML; high SI on short and long TE in myelofibrosis and CLL; high SI on short TE and high to moderately high SI on long TE in MM; and low SI on short and long TE in AA. Quantitative analysis of 33 patients showed high sensitivity and specificity in AA (81% and 94%, respectively) and moderate sensitivity and high specificity in MM (61%, 88%). CML and MDS were similar with low sensitivities (40%, 41%) and high specificities (80%, 78%). Differential diagnosis between CML and MDS was difficult using STIR with the double echo time method.

Adolescent↗
PubMedDate not supplied

Pharmacokinetics of fluconazole in immune-compromised children with leukemia or other hematologic diseases.

STUDY OBJECTIVE: To describe the pharmacokinetics of fluconazole in immune-compromised children with leukemia or other hematologic disease. DESIGN: Prospective. SETTING: Children's Health Care-Minneapolis hematology/oncology inpatient ward and outpatient clinic. PATIENTS: Ten immune-compromised children (mean +/- SD age 7.4 +/- 4.0 yrs, weight 31.6 +/- 25.9 kg) with leukemia or other hematologic disease. INTERVENTIONS: Serum was sampled before and after a single 6-mg/kg intravenous dose and after seven oral 3-mg/kg doses of fluconazole. MEASUREMENTS AND MAIN RESULTS: Mean (SD) pharmacokinetics were distribution half-life 1.67 (1.25) hours, elimination half-life 15.62 (3.21) hours, total body clearance 0.63 (0.19) ml/min/kg, volume of distribution for the central compartment 0.56 (0.10) L/kg, volume of distribution at steady state 0.77 (0.12) L/kg, absorption half-life 0.41 (0.26) hour, and oral bioavailability 0.92 (0.09). Volume of distribution for the central compartment was highly correlated with body surface area (r2 = 0.891) and weight (r2 = 0.949). Volume of distribution at steady state correlated with body surface area (r2 = 0.986), and total body clearance correlated with body surface area (r2 = 0.867). CONCLUSIONS: Fluconazole elimination was well described using a two-compartment model. Oral absorption was rapid and nearly complete. Children have a larger volume of distribution for the central compartment and faster elimination rate than adults. Body surface area and weight are important factors in determining pharmacokinetics in these patients.

Absorption↗
PubMed2006

Laparoscopic splenectomy for hematologic diseases: a preliminary analysis performed on the Italian Registry of Laparoscopic Surgery of the Spleen (IRLSS).

BACKGROUND: The Italian Registry of Laparoscopic Surgery of the Spleen (IRLSS) was developed to provide at the national level an informative tool useful for performing multicenter studies in the field of spleen laparoscopic surgery. In this first study analyzing the IRLSS data, a cohort of patients with hematologic diseases was retrospectively investigated for potential predictive parameters that could affect the outcome of laparoscopic splenectomy. METHODS: A total of 309 patients who underwent laparoscopic splenectomy for hematologic diseases in 17 Italian centers (between February 1, 1993, and September 30, 2004) were entered in the IRLSS. Their records were analyzed retrospectively by the Student's t-test, chi-square, and logistic regression. RESULTS: The mean operative time was 141 min (range, 30-420 min). Conversion was necessary in 21 cases (7%), and approximately 1 accessory spleen in 25 patients (9%) was found. The mean spleen weight was 1191 g (range, 85-4,500 g). Perioperative death occurred in two cases (0.6%). No complications were experienced by 253 patients (81.9%), who had a mean hospital stay of 5.4 days (range, 2-30 days). Overall morbidity occurred in 56 patients (18.1%), mainly associated with transient fever (n = 22), pleural effusion (n = 13), and actual or suspected hemorrhage (n = 12), requiring a reintervention for 7 patients. Multivariate analysis found that body mass index (p = 0.024) and clinical indication (p = 0.004) were independent predictors for surgical conversion. The clinical indication was almost significant as an independent predictor for the occurrence of postoperative complication (p = 0.05). CONCLUSIONS: This first study analyzing the IRLSS data shows that laparoscopic splenectomy may represent the gold standard treatment for hematologic diseases with normal-size spleen. The low morbidity and mortality rate suggests that laparoscopic splenectomy can be successfully proposed also for splenomegaly in hematologic malignancies.

Adolescent↗
PubMedDate not supplied

Efficacy of sulbactam/cefoperazone for the treatment of infections in patients with hematologic diseases.

The efficacy and safety of sulbactam/cefoperazone (SBT/CPZ) was studied in 94 patients with severe infections and concomitant hematologic diseases. All of the study patients were included in the evaluation for safety, and 76 cases were evaluable for efficacy. Clinical efficacy was excellent in 13 cases (17.1%), good in 27 cases (35.5%), fair in seven cases (9.2%), and poor in 29 cases (38.2%). The bacteriologic eradication was 66.7% for Gram-negative bacilli and 50.0% for Gram-positive bacteria. The efficacy rate for neutropenic patients with counts less than 50 mm3 and 100 mm3 were 47.5 and 42.9%, respectively. Efficacy in patients for whom other antibiotic therapy before treatment with SBT/CPZ had been ineffective was 46.2%. Side effects were reported in one case (1.1%), and abnormal serum liver tests in five cases (5.3%); both returned to normal after discontinuation of the study medication. SBT/CPZ was an effective antibiotic for the treatment of severe infections in the presence of concurrent hematologic diseases.

Adolescent↗
PubMed1991

[Histological differences between sternal and iliac bone marrow in various hematological diseases].

Even in normal state, hematopoietic activity is different in terms of the site of bone marrow as well as age. The activity may also differ with bone marrow site in various hematological diseases. We chose 32 cases with various hematological diseases and examined their bone marrow histologically at two different sites (sternum and posterior iliac crest) at almost the same time. We studied the histological differences in 5 cases of malignant lymphoma, 15 cases of aplastic anemia, 8 cases of myelodysplastic syndrome, 2 cases of myeloproliferative syndrome and 2 cases of acute leukemia. In malignant lymphoma, the iliac marrow was slightly hypoplastic compared with sternal marrow but there were no differences in the components of hematopoietic cells, that were thought to reflect normal hematopoietic activity for their respective ages. In 5 among 15 cases of aplastic anemia, nodular hyperplastic areas were observed in sternal marrow even though iliac marrow was severely hypoplastic. The presence of a nodular hyperplastic area is useful for differential diagnosis between aplastic anemia and refractory anemia, because such a histological change was not observed in the cases of refractory anemia. In myeloproliferative syndrome and acute leukemia, there were no differences between sternal and iliac marrow. They were hyperplastic and had almost the same hematopoietic components.

Adult↗
PubMed1993

Bone marrow transplantation for hematological diseases in Hokkaido--June 1985 to December 1991.

Bone marrow transplantation (BMT) was started in Hokkaido in 1985. In the present report we have reviewed the clinical outcome of patients treated with BMT for hematological diseases in Hokkaido. Fifty-eight allogeneic and 19 autologous transplants were registered by December 1991. The underlying diseases consisted of 47 leukemias, 14 lymphomas, 10 aplastic anemias and six myelodysplastic syndromes. Among the allogeneic BMT cases, 55 were human leucocyte antigen (HLA) identical and three were mismatched. Among the autologous BMT patients, two received their marrow purged with 4-hydroperoxycyclophosphamide and five, with monoclonal antibodies and complements. The conditioning regimens used for malignancies were chiefly cyclophosphamide (CY) plus total body irradiation, or busulfan plus CY. In many cases, cytokines were used for rapid recovery of decreased leukocytes. Engraftment was observed in 50 out of 52 evaluated allogeneic and 18 out of 19 autologous transplants. Ten allogeneic patients suffered from severe acute graft-versus-host diseases (GVHD), and extensive chronic GVHD appeared in 16 patients. Relapses were observed in four cases of allogeneic BMT and six of autologous BMT. The major complications were interstitial pneumonitis (IP) and severe infections. Long-term survival rates were almost 60% in both allogeneic and autologous transplants. Mild acute GVHD and limited chronic GVHD increased the survival rates. The results indicated that substantial problems such as GVHD, IP and relapses must be controlled in the near future for an improved outcome to be made possible.

Adolescent↗
PubMed2006

Flow cytometric patterns in blood from dogs with non-neoplastic and neoplastic hematologic diseases using double labeling for CD18 and CD45.

BACKGROUND: In dogs, flow cytometry is used in the phenotyping of immunologic cells and in the diagnosis of hemic neoplasia. However, the paucity of specific antibodies for myeloid cells and B lymphocytes and of labeled antibodies for multicolor techniques limits the ability to detect all leukocyte subpopulations. This is especially true for neoplastic and precursor cells. CD18 and CD45 are expressed on all leukocytes and are involved in cell activation, and together could be useful in helping determine cell lineage. OBJECTIVES: The purpose of this study was to double label canine blood for CD18 and CD45 and to use the differential expression of antigens to identify leukocyte populations in dogs with non-neoplastic and neoplastic hematologic diseases. METHODS: A template was developed using blood samples from 10 clinically healthy dogs and a back-gating technique. Differential leukocyte counts obtained with the template were compared with those obtained by manual and automated methods on blood samples from 17 additional healthy dogs. Blood samples obtained from 9 dogs with non-neoplastic (reactive) hematologic diseases and 27 dogs with hemic neoplasia were double stained for CD18 and CD45 using mouse anticanine CD18 monoclonal antibody (mAb) plus phycoerythrin-conjugated rat anticanine CD45 mAb and fluorescein isothiocyanate-conjugated rabbit antimouse IgG. Hemic neoplasms were diagnosed by cell morphology, and immunophenotypic and cytochemical markers. RESULTS: With the double label, neutrophils, eosinophils, monocytes, and T- and B-lymphocytes were identified. In reactive disorders, a population of activated neutrophils with high CD45 and CD18 expression was detected. In hemic neoplasia, cell lineage was easily determined, even in acute leukemia. CONCLUSIONS: Double labeling for CD18/CD45 may be useful as a screening method to evaluate hematologic diseases and help determine cell lineage, and to aid in the selection of a panel of antibodies that would be useful for further analysis.

Animals↗
PubMed2002

[A pilot study of G-CSF mobilized allogeneic bone marrow cells plus peripheral blood stem cells transplantation for malignant hematological diseases].

OBJECTIVE: To study the feasibility and clinical outcome of granulocyte-colony stimulating factor (G-CSF) mobilized allogeneic bone marrow (G-BM) plus G-CSF mobilized peripheral blood stem cells (G-PBSC) transplantation for malignant hematological disease. METHODS: G-BM plus G-PBSC transplantation was conducted between siblings or between parent and child. G-CSF was injected hypodermically into 14 stem cell donors 5 micro g x kg(-1) x d(-1) for 4 approximately 6 days. On the 3rd day after beginning of G-CSF injection bone marrow blood was extracted from the posterior superior iliac spine of the donors; on the 4th day G-PBSCs were collected. The collected stem cells were transplanted immediately into the bodies of the 14 patients with hematological disease, including acute myelocytic leukemia, acute lymphocytic leukemia, multiple myeloma, myelodysplastic syndrome and severe aplastic anemia. The clinical outcomes, including the rate of engraftment, speed of engraftment, and the occurrence of acute graft versus host disease (aGVHD) and chronic graft versus host disease (cGVHD) were investigated. RESULTS: All patients got endurable engraftment. The median time of recovery of neutrophil count to >or= 1 x 10(9)/L was 14 days (range 12 approximately 18 days). The median time to achieve a platelet count >or= 20 x 10(9)/L without transfusion was 13 days (range 11 approximately 65 days). Seven of the 14 patients developed aGVHD, 4 being in the grade I and 3 being in grade II. Three of the evaluable 9 patients developed extensive chronic GVHD. Two patients died of relapsed leukemia. One patient received secondary PBSCT from hte same donor because of ill engraftment and developed grade IV GVHD and died. The other 11 patients were still alive and event-free with a median follow-up duration of 455 days (range 84 approximately 715 days). CONCLUSION: An effective treatment for malignant hematological disease, G-CSF mobilized allogeneic bone marrow plus peripheral blood stem cells transplantation provides rapid and sustained engraftment without increase in incidence of aGVHD and cGVHD.

Adolescent↗
PubMedDate not supplied

Laparoscopic splenectomy in the management of benign and malignant hematologic diseases.

OBJECTIVES: The use of laparoscopy to treat malignant hematological diseases is not completely accepted. Our aim was to analyze operative and postoperative results of laparoscopic splenectomy performed for benign versus malignant hematological disorders. METHODS: Between 1994 and 2003, 76 consecutive patients underwent laparoscopic splenectomy. The first 38 cases were performed by using an anterior approach, whereas in the remaining 38 cases a semilateral position was used. RESULTS: Baseline characteristics showed that patients with malignant diseases were significantly older (56.9 vs 32.6 years, P < 0.001). Seventy-two (94.7%) procedures were completed laparoscopically. Conversion was required in 4 cases (5.2%). Mean operative time was 138.5 minutes for benign and 151.0 minutes for malignant diseases, (P > 0.05, ns). The hand-assisted technique was used in 3 patients with massive splenomegaly. Pathologic features showed that spleen volume was higher in patients with malignant diseases (mean interpole diameter 18.1 cm vs 13.7 cm, P < 0.001). Massive splenomegaly (interpole diameter over 20 cm, weight over 1000 g) was present in 13 patients (17.1%); 9 had malignant diseases. Overall perioperative mortality was 1.3% and major postoperative complications occurred in 6 patients (7.8%). Postoperative splenoportal partial thrombosis was identified in 9.7% of patients. CONCLUSIONS: Laparoscopic splenectomy is a well-accepted, less-invasive procedure for hematological disorders. Neoplastic diseases or splenomegaly, or both, do not seem to limit the indications for a minimally invasive approach after the learning curve.

Adolescent↗