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M Uppenkamp

Publications and source records attributed to M Uppenkamp.

At least 19 recordsLinked to original sources

[Therapy of hypercalcemia with ibandronate in case of acute renal failure].

Hypercalcaemia is a common complication of malignancies associated with bone destruction. Besides, benign diseases as sarcoidosis or hyperparathyroidism may lead to hypercalcaemia. The main principles of modern therapy contain a forced diuresis as well as the application of bisphosphonates. Latter substances bear the danger of developing a renal insufficiency. Here, we report the case of a female patient, suffering from primary hyperparathyroidism with severe hypercalcaemia and calcium levels up to 6 mmol/l, who developed acute renal failure. We treated the patient with forced diuresis and repeated infusions of ibandronate (5 x 6 mg ibandronate). Even if lowering the serum levels of calcium only for a short time after each application, yet we could improve renal function by these means. Only after performing a parathyroidectomy, we could see a sustained decline of calcium levels. This case report supports the results of other publications, that have reported the missing nephrotoxic effect of ibandronate compared to other bisphosphonates.

Acute Kidney Injury↗

High bone-binding capacity of ibandronate in hemodialysis patients.

Bisphosphonates are a potential therapy for osteoclast-mediated bone disease, such as renal osteodystrophy. This study evaluated ibandronate bone-binding in patients with secondary hyperparathyroidism and renal osteodystrophy and examined whether there is a correlation with bone metabolism parameters. Sixteen patients with end-stage renal disease and secondary hyperparathyroidism receiving regular hemodialysis were recruited to this 12-week trial. Intravenous ibandronate 2 mg was administered for 5 min every 4 weeks directly after hemodialysis. Ibandronate levels were measured 15 min after infusion and at trough levels before the next hemodialysis. Serological markers of bone metabolism were also measured. After the first infusion, the peak ibandronate level was 154 +/- 75.1 ng/ml and the trough level was 2.7 +/- 1.7 ng/ml. At week 12, peak and trough ibandronate levels were 164.8 +/- 89.9 ng/ml and 3.2 +/- 2.6 ng/ml, respectively. Ibandronate bone uptake was 98.0% at first application and 98.4% at week 12. In patients with remaining diuresis, ibandronate urine excretion was < 0.001% of the administered dose. There was no correlation of ibandronate bone-binding with parameters of osteoclast activity or parathyroid hormone (PTH). The correlation with markers of osteoblast activity was significant but weak. Ibandronate had a bone-binding capacity of approximately 98% in hemodialysis patients. After repeated dosing ibandronate bone-uptake remained stable and was independent of osteoclast activity or PTH levels. Due to the high bone-binding of ibandronate in these patients, a 2 mg dose of intravenous ibandronate is equivalent to a 4-5 mg dose of ibandronate in patients with normal renal function.

Adult↗

Therapy of IgA nephropathy with mycophenolate mofetil--report of 3 cases.

Mycophenolate mofetil is an immunosuppressive agent in transplantation which inhibits the purin neogenesis. Proliferating lymphocytes are suppressed and antibody production is decreased. Many cases of successful therapy in different kidney diseases are reported, such as diffuse proliferative lupus nephritis, pauci-immune necrotizing glomerulonephritis, focal segmental glomerular sclerosis and IgA nephropathy. We report 3 patients with IgA nephropathy who were treated with mycophenolate mofetil for more than 1 year. In all patients, proteinuria decreased significantly and the renal function remained stable. In 2 patients, kidney biopsy was repeated after 12 months and 18 months, respectively. There were no histological signs of progression of the disease. Two patients developed infections during treatment. One patient had a pneumonia, and a second patient an infection with varizella zoster. Based on our data, mycophenolate mofetil can be a potential treatment of IgA nephropathy. Further controlled studys are warranted to investigate the role of mycophenolate mofetil in IgA nephropathy.

Adult↗

Gamma-irradiation of blood products following autologous stem cell transplantation: surveillance of the policy of 35 centers.

Blood products should be irradiated during allogeneic stem cell transplantation and before performing autologous stem cell harvest for prevention of acute transfusion-associated graft-versus-host disease (TA-GVHD). Usually, irradiation of all blood products is continued lifelong in the allogeneic setting. Up to now, no broadly accepted rules exist concerning autologous stem cell transplantation. We present here the results of an inquiry sent to 47 German transplantation centers regarding the transfusion policy following autologous stem cell transplantation. The results of 35 answering centers are included. Ten out of 35 centers offer irradiated blood products lifelong to their patients, mainly for the prevention of mistransfusion of non-irradiated blood components to allogeneic recipients. Twenty-two out of 35 centers administer irradiated blood products for a special time span after autologous stem cell transplantation. In most centers, this time span is from 3 to 6 months. Only few centers (4/35) expand this time span to 1-2 years after transplantation. A minority of centers (3/35) gave non-irradiated blood products to all of their patients or to patients not suffering from acute leukemia or after total body irradiation (TBI) containing preparative regimens. Most centers (19/35) deliver irradiated blood products irrespective of the conditioning regimen. Fifty-three percent of the centers decide to donate irradiated blood products not depending on immunological reconstitution. But in most centers some kind of hematological reconstitution is a major criterion for termination of irradiated blood products. Sixty-four percent of the centers made no difference in transfusion policy in regard to the underlying disease. No center experienced cases of proofed TA-GVHD. Guidelines should be worked out concerning transfusion policy after autologous stem cell transplantation.

Blood↗

Classification of malignant lymphoma.

Over the past 30 years different lymphoma classifications were used world-wide. In Europe, the Kiel classification of K. Lennert dominated the scene, it was updated 1988 and 1992. In North America the Working Formulation, primarily designed to be an instrument for translation of one classification into the other, was predominantly used. Both classifications, however, showed little correspondence with each other, which made it difficult to compare the results of randomized clinical trials on both sides of the Atlantic Ocean. In 1994 the Revised European American Lymphoma (R.E.A.L.) classification was proposed by the International Lymphomas Study Group (ILSG). In contrast to the existing classifications it abandoned the grading of malignancies. The ILSG focused on diagnostic reproducibility and on the definition of distinct clinical-pathological lymphoma entities. Similar to the Kiel classification the new classification scheme was based on cell lineage (T- and B-cell origin) and cell differentiation (precursor and mature lymphomas) and included a number of distinct extranodal lymphomas. The new WHO classification is basically identical with the R.E.A.L. scheme and exhibits only minor changes. The WHO classification includes all hematopoietic and lymphoid neoplasms. It represents the first generally accepted classification, providing hematologists and oncologists with a solid diagnostic basis for therapeutic decisions.

Europe↗

Monoclonal antibody therapy with CAMPATH-1H in patients with relapsed high- and low-grade non-Hodgkin's lymphomas: a multicenter phase I/II study.

CAMPATH-1H (CP-1H) is a humanized monoclonal antibody directed against the CD52 antigen with promising therapeutic effects in patients with small cell lymphocytic non-Hodgkin's lymphomas (NHL) of B- and T-cell type. We report about the response and toxicity of CP-1H in 18 patients with B-cell NHL who were treated in four clinical centers in Germany. Sixteen patients suffered from a low-grade and two from a high-grade NHL. All patients had received chemotherapy before and had either relapsed or were refractory to conventional therapy. Two patients received CP-1H in a dose-range finding trial once weekly and 16 patients as a fixed dose of 30 mg three times weekly. Of 18 patients, 8 (44%) achieved a clinical response, 2 (11%) had stable disease, and 5 (28%) had progressive disease. Four patients could not be evaluated for response because of death (two patients) and serious adverse events (two patients). All patients with response to CP-1H had a low-grade NHL. Nonhematological toxicity was severe in two patients who suffered from WHO grade III/IV bronchospasm. Common acute adverse events (WHO grade I-III) included fever, chills, rigor, urticaria, nausea, and vomiting. Eleven patients suffered from bacterial or viral infections; some had recurrent infections. A total of 12 different infections were reported. The most frequent infections were caused by herpesvirus (seven patients). Hematological toxicity included thrombocytopenia in four and lymphocytopenia in seven patients. Although the antibody is humanized, the nonhematological toxicity was substantial and probably due to a cytokine release syndrome. Prophylactic treatment of the side effects is strongly recommended for patients treated either with CP-1H alone or in combination with chemotherapy.

Aged↗

[Complications after high dose therapy and autologous stem cell transplantation. Retrospective study of an unselected patient sample].

BACKGROUND: High-dose therapy (HDT) with autologous blood stem cell transplantation (ASCT) has become the therapy of choice for patients with specific hematologic neoplasms. Although pancytopenia after HDT with stem cell support is of relatively short duration, complications may be severe and life-threatening. In unselected patients with hematologic and solid tumor malignancies, only few data have been published regarding complications. We therefore analyzed the rate of infection and toxicity in patients with different neoplasms undergoing HDT and ASCT. PATIENTS AND METHODS: From 6/96 to 12/99 42 patients received 54 HDT and ASCT (nine tandem transplants and one triple transplant). The median age was 55 years (range 25-74 years) with equal sex distribution. 30 patients suffered from hematologic malignancies and twelve from solid tumors. RESULTS: Infections were the major cause for complications followed by mucositis, pain and diarrhea. In four patients a positive cytomegalovirus polymerase chain reaction (CMV-PCR) was detected. In two patients this positive test result was accompanied by clinical symptoms of CMV infection. One patient developed lung fibrosis due to busulfan (WHO 4 degrees) and additionally a veno-occlusive disease (VOD) of the liver (WHO 4 degrees). Two patients (4%) died due to CMV pneumonia and multiple organ failure after idiopathic pneumonia, respectively. Four patients developed secondary neoplasms (two patients myelodysplastic syndromes, two patients solid tumors). Three of them had been heavily pretreated. We further analyzed whether the following parameters had an influence on the rate of complications: tumor diagnosis (hematologic vs. solid), number of pretreatment protocols (< 2 vs. > or = 2), CD34+ cell count (< median CD34+ cell count vs. > or = median CD34+ cell count), age (< or = 55 years vs. > 55 years), mucositis (WHO 1-2 degrees vs. 3-4 degrees) and conditioning regimen (myeloablative vs. myelosuppressive). The infection rate was higher in patients receiving myeloablative therapy compared to patients with myelosuppressive conditioning and the platelet count recovery was slower. In patients receiving a higher CD34+ cell count, time until platelets reached > 50/nl was shorter than in patients with a lower CD34+ cell count. Patients with > or = 2 pretreatment protocols had a higher infection rate than patients with < 2 pretreatments. Patients suffering from severe mucositis (WHO 3-4 degrees) exhibited a slower platelet recovery and a higher infection rate. No difference was noted in the complication rate for the other parameters (tumor diagnosis, age). CONCLUSION: Complication rate and mortality in this heterogeneous patient group were not different from the data of other authors describing selected patients receiving a uniform conditioning regimen or having a distinct disease. The complication rate is influenced by the number of pretreatment protocols, conditioning regimens and the number of transplanted CD34+ cells.

Adult↗

[Kidney involvement in hypocomplementemic urticaria-vasculitis syndrome--a simulated systemic lupus erythematosis].

We report a case of hypocomplementemic urticarial vasculitis (HUV). The clinical course was characterized by urticaria, angio-edema, pericarditis, joint pain and conjunctivitis. The laboratory findings revealed moderate proteinuria, erythrocyturia, normal renal function, normotension, reduction of C3, C4 and C1q complement with elevated C1q antibodies. Antinuclear antibodies were inconstantly positive. Renal biopsy showed a mild form of membranous glomerulonephritis. Despite intensive therapeutic measures (dapsone, immunosuppressives and immunoglobulins), relapses of urticaria occurred frequently. HUV is probably a minor form of systemic lupus erythematosus.

Adult↗

CD34+ cell enrichment for autologous peripheral blood stem cell transplantation by use of the CliniMACs device.

Several devices for selection of CD34+ peripheral blood stem cells (PBSC) have been used during the last years for reducing tumor cell contamination of the graft. The new CliniMACS system (magnetic-activated cell separation system by Miltenyi Biotech GmbH, Bergisch-Gladbach, Germany) was recently approved for clinical use in Europe. To evaluate its purging efficiency and engraftment data in the autologous transplant, PBSC from 28 adult patients with various malignant diseases (non-Hodgkin's lymphoma, n = 17; chronic lymphocytic leukemia, n = 5; multiple myeloma, n = 4; acute lymphocytic leukemia, n = 1; medulloblastoma, n = 1) were mobilized by chemotherapy and granulocyte colony-stimulating factor (G-CSF) (10 microg/kg per day). Thirty leukapheresis products from 28 patients with a median of 4.4 x 10(8) nucleated cells/kg body weight (bw)(range 0.6-10.8 x 10(8)/kg bw) and a median of 7.1 x 10(6) CD34+ cells/kg bw (range 2.8 to 18.8 x 10(6)/kg bw) were selected using the Cobe spectra cell separator (Cobe BCT Inc., Lakewood, CO). After the CliniMACS procedure, the median yield of CD34+ selected cells was 4.5 x 10(6)/kg (range 2.2-11.1 X 10(6)/kg bw) with a median recovery of 69.5% (range 46.9-87.3%) and a median purity of 97.7% (range 89.4-99.8%). The procedure did not alter viability of selected cells, which was tested by propidium iodide staining. So far, purified PBSC were used for autologous transplantation in 15 out of 28 patients after total body irradiation and/or high-dose chemotherapy. Median time to reach an absolute neutrophil count > 500/microl was 12 days (range 10-18 days), platelet recovery >50,000/microl occurred at day + 16 (range 11-22). With a median follow-up time of 12 months (range 3-19), 5 patients died of relapse. We confirmed the feasibility and safety of the CliniMACS CD34+ cell enrichment procedure in adult patients with autologous PBSC transplantation.

Adult↗

Renal complications of high-dose chemotherapy and peripheral blood stem cell transplantation.

Following bone marrow transplantation, acute renal failure and proteinuria are common complications with a high mortality, particularly in patients requiring hemodialysis. Incidence, potential predisposing factors, and outcome of acute renal complications in patients with hematological malignancies receiving autologous peripheral blood stem cell transplantation were prospectively studied in 53 patients. Eight patients developed acute renal failure. Three of them required hemodialysis. Of all patients with acute renal failure, only those requiring hemodialysis died, due to nonrenal causes. Only 1 of the 45 patients without renal failure died. Mild proteinuria of predominantly tubular origin occurred in 16 patients, in 3 with and in 13 without acute renal failure. As predisposing factors for acute renal failure were identified: renal hypoperfusion due to systemic inflammatory response syndrome, sepsis or septic shock, and combined administration of nephrotoxic drugs. Especially those patients receiving high numbers of nephrotoxic drugs in combination with renal hypoperfusion were likely to develop acute renal failure. These results suggest that patients receiving high-dose chemotherapy and autologous peripheral blood stem cell transplantation have a low risk of developing acute renal failure and proteinuria.

Acute Kidney Injury↗

Chédiak-Higashi-Steinbrinck syndrome (CHS) in a 27-year-old woman--effects of G-CSF treatment.

Chédiak-Higashi-Steinbrinck syndrome (CHS) is a rare autosomal recessive disorder which is usually lethal in early childhood. Diagnostic hallmark is the occurrence of giant inclusion bodies in peripheral leukocytes and their bone marrow precursors. We report on a 27-year-old female patient who was admitted for treatment of a skin abscess. She recovered after intravenous antibiotic treatment and surgical incision. Hematological investigation was initiated because of a persisting neutropenia of 15%, with a leukocyte count initially in the normal range but subsequent leukopenia. Case history revealed recurrent skin infections from childhood on, regularly requiring surgical intervention. One year prior to admission a neuropathy had been diagnosed, while a partial albinism had been known for years. Microscopic examinations of peripheral blood and bone marrow aspirate smears were diagnostic for CHS. Additionally, a secondary antibody deficiency was found. Normalization of the white blood cell count, including the differential count, was observed following initiation of G-CSF treatment. Functional assessment of phagocytosis and oxidative burst activity of granulocytes revealed normal results before and after stimulation with G-CSF, however, natural killer cell activity was only weak, with slight improvement after G-CSF treatment in vivo. Cytogenetic analysis showed a normal female karyotype. Although the haploidentical brother of the patient may serve as an allogeneic stem cell donor, transplantation has been postponed because of further deterioration of her already existing CHS-specific neurological impairment. Nevertheless, while receiving G-CSF maintenance treatment our patient experienced no further infectious episodes within 6 months after diagnosis of CHS.

Adult↗

Unique alterations of thyroid function parameters after i.v. administration of alkylating drugs (cyclophosphamide and ifosfamide).

Alkylating drugs (cyclophosphamide and ifosfamide) have been in clinical use for the treatment of malignant diseases in the past. They are most useful anticancer agents and cyclophosphamide is also widely used for its immunosuppressive properties. However the effect of alkylating drugs on thyroid hormone parameters have not been evaluated so far. Three groups of patients were prospectively evaluated: Group I: 15 patients with Wegener's granulomatosis and 4 patients with severe scleritis received a single dose cyclophosphamide (15 mg/kg bw/day) and 250 mg prednisone i.v. Group II: 9 patients with malignant lymphomas were treated according to the IMVP 16-protocol. Patients received daily ifosfamide 1000 mg/m2 from day 0 to 4 and vepesid from day 0 to 2. Patients did not receive corticosteroids additionally. Group III: 6 patients with a relapse of malignant lymphomas received ifosfamide 1.500 mg/m2/day from day 0 to 4 i.v. and dexamethasone 40 mg/m2 as well as ara-c and etoposid. All patients received mesna to prevent hemorrhagic cystitis and odansetran or metoclopramide as antiemetic drugs. Alkylating drugs were given as a one hour infusion. Thyroid hormone parameters were determined before and on day 1, 2, 3, 4 after drug administration. We observed a significant increase in T4 and fT4 concentrations and a concomitant fall in TSH in either group one day after the administration of alkylating drugs. The effect was most pronounced in group III: T4 increased from 113 +/- 8 nmol/L to 175 +/- 8 (normal: 58-154) and fT4 from 14.0 +/- 0.8 to 24.8 +/- 2.5 pmol/L (normal 10-25). TSH dropped from 1.27 +/- 0.16 to 0.33 +/- 0.07 mU/L (normal 0.3-4). All changes were significant: p < 0.001. Two of the six patients displayed biochemical hyperthyroidism. Also reverse T3 increased significantly. Two days after drug administration a gradual normalization occurred. However, T3, Tg, TBG, Transthyretin and albumin levels did not change throughout the study period. One patient with coexisting hypothyroidism, who received his last thyroxine substitution therapy one day before the administration of cyclophosphamide (as in group I), also demonstrated an increase in T4, fT4 and rT3 and a fall in TSH concentrations. I.v. administrations of cyclophosphamide and ifosfamide induce a transient increase in T4 and fT4 concentrations and a concomitant fall of TSH in the presence of normal Tg, T3 and thyroid binding protein concentrations. These data suggest, that the changes are not due to a release of thyroid hormones from the thyroid itself, but is likewise related to a release of thyroxine from cellular pools such as the liver.

Adult↗

Harvesting of peripheral blood progenitor cells with different programmes of discontinuous flow systems.

BACKGROUND AND OBJECTIVES: From a technical point of view two problems arise during the collection of peripheral blood progenitor cells (PBPC): first, the volume of the apheresis product is often large, requiring volume reduction prior to cryopreservation. Second, the platelet (PLT) loss due to the harvesting of the buffy coat is an unwanted side effect. With respect to these problems, the present study was designed to compare programs for PBPC collection with discontinous-flow cell separators. MATERIALS AND METHODS: Three different protocols for PBPC harvesting were investigated in 32 patients with malignancies. In the first protocol, the blood cell separator Haemonetics MCS 3p was used. In the second protocol using the same machine, the opening and closure of the stem cell valve was modified in combination with a centri surge to reduce the PLT loss. The MCS+ device with another configuration of the valves was used in the third protocol. PBPC were mobilised by chemotherapy plus cytokine administration or application of growth factor alone. Blood counts and CD34 antigen-expressing cells were determined before apheresis and in the PBPC product. Colony-forming unit granulocyte/macrophage (CFU-GM) were determined in the apheresis product. RESULTS: 55 PBPC collections were carried out with a median end product volume of 105 ml. The median counts for CD34+ antigen-expressing cells and CFU-GM were 1.5x10(6 )and 2.5x10(4)/kg body weight, respectively. PLT loss was significantly lower in protocol III. CONCLUSION: The study reported here revealed that PBPC could be easily collected in a reduced product volume by the intermittent-flow cell separators. No additional centrifugation prior to cryopreservation was necessary to remove the plasma from the apheresis product. Patient's PLT loss was reduced by the centri surge technique but this still has to be improved.

Anticoagulants↗

[POEMS syndrome. A rare variant of osteosclerotic multiple myeloma with polyneuropathy, organomegaly, endocrinopathy, M-gradient and skin lesions].

BACKGROUND: A rare multi-organ involvement in plasma-cell dyscrasias has been named POEMS-syndrome: it is a synopsis of monoclonal gammopathy (M-gradient), osteosclerotic bone lesions, peripheral polyneuropathy, organomegaly, endocrinopathy and skin lesions. CASE REPORT: A patient is presented who had a classical manifestation of this disease known mainly in Japan. A monoclonal IgA-lambda-gammopathy was determined as cause of a gradually progressive polyneuropathy. The patient had a hypergonadotropic hypogonadism, hyperprolactinaemia, and sclerotic bone lesions. In addition, he showed a changing organomegaly, and hyperpigmentation of the skin. CONCLUSION: As yet, aetiology and pathophysiology are not fully understood. Irradiation or surgical resection of one or several osteosclerotic bone lesions may improve the polyneuropathy or may even lead to a complete remission of all symptoms. Thus, monoclonal immunoglobulins should be searched for in any unclear polyneuropathy, as should be for other symptoms of the POEMS-syndrome.

Diagnosis, Differential↗

Thrombopoietin serum concentration in patients with reactive and myeloproliferative thrombocytosis.

We wished to test whether thrombopoietin (TPO) is entirely regulated by receptor binding or if other factors may play a role in the mechanism of TPO regulation. Therefore, we analyzed the TPO serum levels in 43 patients with reactive (secondary) thrombocytosis and in 37 with myeloproliferative thrombocytosis. Thrombocytosis was defined as a platelet level greater than 440 x 10(9)/l. Forty-two patients (98%) with reactive thrombocytosis had high concentrations of IL-6 correlating with elevated C-reactive protein levels. Twenty-three patients (53%) in this group had TPO serum concentrations of more than 300 pg/ml (normal: below 300 pg/ml). Only nine patients (24%) with myeloproliferative thrombocytosis had TPO serum levels above normal range, whereas 28 patients (76%) had normal levels of TPO. No correlation between the TPO serum levels and the concentrations of IL-6 or EPO was established. The other investigated thrombopoietic cytokines (IL-3, IL-11, GM-CSF) were unmeasurable; therefore, a correlation could not be assessed. We conclude that TPO concentrations are not strictly inversely related to platelet count. TPO serum levels are elevated especially in a considerable percentage of patients with reactive thrombocytosis, arguing for the existence of additional mechanisms of TPO regulation.

Adolescent↗

CAMPATH-1H monoclonal antibody in therapy for previously treated low-grade non-Hodgkin's lymphomas: a phase II multicenter study. European Study Group of CAMPATH-1H Treatment in Low-Grade Non-Hodgkin's Lymphoma.

PURPOSE: CAMPATH-1H is a human immunoglobulin G1 (IgG1) anti-CD52 monoclonal antibody (MAb) that binds to nearly all B-cell and T-cell lymphomas. We report here the results of a multicenter phase II trial of CAMPATH-1H in patients with advanced, low-grade non-Hodgkin's lymphoma (NHL) who were previously treated with chemotherapy. PATIENTS AND METHODS: Fifty patients who had relapsed (n=25) after or were resistant (n = 25) to chemotherapy were treated with CAMPATH-1H 30 mg administered as a 2-hour intravenous (i.v.) infusion three times weekly for a maximum period of 12 weeks. RESULTS: Six patients (14%) with B-cell lymphomas achieved a partial remission (PR). Patients with mycosis fungoides appeared to respond more frequently (50%; four of eight patients, which included two complete remissions [CRs]). Lymphoma cells were rapidly eliminated from blood in 16 of 17 patients (94%). CR in the bone marrow was obtained in 32% of the patients. Lymphoma skin lesions disappeared completely in four of 10 patients and partial regression was obtained in three patients. Lymphadenopathy and splenomegaly were normalized in only 5% and 15% of patients, respectively. Lymphopenia (< 0.5 x 10(9)/L) occurred in all patients. World Health Organization (WHO) grade IV neutropenia occurred in 14 patients (28%). Opportunistic infections were diagnosed in seven patients and nine patients had bacterial septicemia. Death related to infectious complications occurred in three patients. CONCLUSION: CAMPATH-1H had a significant but limited activity in patients with advanced, heavily pretreated NHL. The most pronounced effects were noted in the blood and bone marrow and in patients with mycosis fungoides. The risk for serious infectious complications needs to be considered for severely ill patients who are evaluated for CAMPATH-1H treatment.

Adult↗

Antibody responses of splenectomized patients with non-Hodgkin's lymphoma to immunization with polyvalent pneumococcal vaccines.

The serum antibody responses of splenectomized patients with non-Hodgkin's lymphoma (NHL) who had been immunized with a polyvalent pneumococcal vaccine (Pneumovax 23) were evaluated by an enzyme-linked immunosorbent assay with the 23-valent pneumococcal vaccine as the antigen. A response to immunization, defined as a twofold-or-higher rise of the prevaccination titer of antibodies against Streptococcus pneumoniae polysaccharide, was elicited in 5 of 11 patients with NHL. No significant difference in the level of antibodies against S. pneumoniae polysaccharide between lymphoma patients and patients who had undergone splenectomy for other reasons was detected (P = 0.83 and 0.87 before and after vaccination, respectively). NHL patients who did not respond to the first immunization received a booster dose of the polysaccharide vaccine. This injection did not increase the pneumococcal-antibody titer significantly (P = 0.7). We conclude that vaccination with pneumococcal polysaccharides in splenectomized patients with NHL elicits an adequate antibody response in 45.4% of the cases and should therefore be administered. Revaccination of the nonresponders does not further increase the pneumococcal-antibody levels.

Adult↗

[Hemolytic anemia as cause of a marked bilirubin increase in primary biliary cirrhosis].

HISTORY AND CLINICAL FINDINGS: 16 years ago a now 53-year-old woman was found to have primary biliary cirrhosis. 5 years later, after bleeding from oesophageal varices, she had a portacaval shunt. For several years she had been taking ursodeoxycholic acid (750 mg daily). Because of steadily increasing jaundice over the past few years she presented for possible liver transplantation. INVESTIGATIONS: There was a discrepancy between the markedly raised serum bilirubin concentration (7.8 mg/dl) and the only slightly raised or normal activities of alkaline phosphatase (247 U/l) and gamma-GT (21 U/l). Further tests confirmed that the patients had not only PBC but also Coombs-negative haemolytic anaemia (haemoglobin 10.7 g/dl, reticulocyte count 122/1000, indirect bilirubin 6.4 mg/dl, haptoglobin not demonstrated, lactate dehydrogenase 316 U/l). She had splenomegaly despite the portacaval shunt. Blood smear revealed spherocytes, but hereditary spherocytosis was not confirmed. TREATMENT AND COURSE: A six-week interruption of taking ursodeoxycholic acid led, as expected, to a rise in the activities of serum alkaline phosphatase and gamma-GT, while haemolysis parameters were not affected. CONCLUSION: Serum bilirubin concentration is a decisive prognostic factor in the course of primary biliary cirrhosis and is therefore of particular relevance for the indication of liver transplantation. The reported case demonstrates the importance of considering other causes of hyperbilirubinaemia.

Alkaline Phosphatase↗