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1.
Vnitr Lek ; 57(2): 214-21, 2011 Feb.
Article in Czech | MEDLINE | ID: mdl-21416862

ABSTRACT

We describe a case of an untreated female patient monitored over 8 years for chronic B-lymphocytic leukaemia (B-CLL). Over the 8 years, the patient has gradually developed severe kidney failure, even though the criteria for B-CLL treatment had not been fulfilled. Kidney biopsy revealed renal damage due to lamda free light chains cast nephropathy as well as an infiltration of renal parenchyma with B-CLL cells. It was not before this biopsy that the presence of monoclonal immunoglobulins has been investigated. Immunofixation identified free monoclonal lamda light chains in the serum and urine. Their serum concentration, quantified by densitometry, was 2.6 g/l and urine concentration was 0.5 g/l. A specific evaluation of free light chains in the serum revealed an extremely high concentration of free X light chains, over 4500 mg/l, and normal concentration of K free light chains, 10 mg/l. The aim of this report is to emphasise that monoclonal immunoglobulin may be present in B-CLL as well as other lymphoprolipherative diseases and that it may cause damage to organs, similar to multiple myeloma or monoclonal gammopathy of undetermined significance. The described case confirms poor prognostic value of monoclonal immunoglobulin free light chains in patients with B-CLL and usefulness of an evaluation of their presence in patients with B-CLL, particularly if the patients have increased creatinine level. The described case also highlights the need for evaluation of the presence of free light chains in the serum of all patients with unclear cause of renal failure.


Subject(s)
Immunoglobulin Light Chains/blood , Leukemia, Lymphocytic, Chronic, B-Cell/complications , Renal Insufficiency/diagnosis , Renal Insufficiency/immunology , Aged , Female , Humans , Kidney/pathology , Renal Insufficiency/etiology , Renal Insufficiency/pathology
2.
Vnitr Lek ; 56(11): 1158-68, 2010 Nov.
Article in Czech | MEDLINE | ID: mdl-21250495

ABSTRACT

UNLABELLED: Monoclonal gammopathy may manifest itself through a range of skin disorders, including plane normolipemic xanthoma and necrobiotic xanthogranuloma. The present paper describes two patients with these cutaneous symptoms. The first has extensive areas of skin affected by flat xanthomas, monoclonal gammopathy with > 10% infiltration of bone marrow with clonal plasmocytes and, according to PET-CT, unclear lymphadenopathy in the retroperitoneal area. The size of this lymphadenopathy (histologically no malignant infiltration and no confirmed infectious aetiology) has not changed significantly over a 4-year follow-up. Repeated PET-CT scans showed decrease in SUV value in this infiltration from 7.5 to 3.8. Four cycles of treatment with a combination of bortezomib, cyclophosphamide and dexamethasone brought neither reduction in monoclonal immunoglobulin nor change to skin morphology. We believe that the abdominal lymphadenopathy is associated with xanthomatosis but have been unable to confirm this unequivocally. The second patient is being followed up for more than 10 years, originally for MGUS, later for asymptomatic multiple myeloma. Last year, painful subcutaneous and cutaneous infiltrates, isolated on an upper limb and more frequent on lower limb, started to occur. These infiltrates are palpable. PET-CT imaging provided an excellent depiction of these infiltrates, showing no pathology on the head, chest and abdomen and no osteolytic foci on the skeleton. CT imaging showed clearly numerous infiltrates in the skin and subcutaneous tissue of lower limbs, particularly both shanks, reaching up to 2 cm in depth. The largest infiltrate, measuring 3.5 by 2 by 10 cm, was identified in the distal dorsal part of the right shank. PET imaging of lower limbs showed distinctly pathological accumulation in all infiltrates described above; the accumulation of glucose in the lower part of the right shank reached 10.0 SUV. CT images of lower limbs showed increased density saturated hypodermis even in the areas where there is no increased accumulation of 18 fluoroglucose. Following 40 Gy irradiation, the size of infiltrate in the radiated area decreased and their soreness ceased. CONCLUSION: PET-CT imaging offered information on extra-cutaneous signs of plane normolipemic xanthomas and provided excellent depiction of the areas of the skin and hypodermis affected by necrobiotic xanthogranuloma. Chemotherapy with cyclophosphamide, bortezomib and dexamethasone brought no reduction in monoclonal immunoglobulin concentration, and no reduction in plane normolipemic xanthomas. Radiotherapy targeted at large foci of xanthogranulomas led to partial regression and ceased infiltrate soreness.


Subject(s)
Monoclonal Gammopathy of Undetermined Significance/complications , Necrobiotic Xanthogranuloma/complications , Xanthomatosis/complications , Humans , Male , Middle Aged , Monoclonal Gammopathy of Undetermined Significance/diagnosis , Monoclonal Gammopathy of Undetermined Significance/therapy , Necrobiotic Xanthogranuloma/diagnosis , Necrobiotic Xanthogranuloma/immunology , Necrobiotic Xanthogranuloma/therapy , Positron-Emission Tomography , Tomography, X-Ray Computed , Xanthomatosis/diagnosis , Xanthomatosis/immunology , Xanthomatosis/pathology , Xanthomatosis/therapy
4.
Vnitr Lek ; 46(11): 750-5, 2000 Nov.
Article in Czech | MEDLINE | ID: mdl-15637889

ABSTRACT

Transplantation of the heart has become an accepted method for the treatment of terminal cardiac failure. Despite obvious advances in the care of patients after trasplantation a number of problems exist. The authors summarize their experience with the long-term follow-up of 100 adult patients with transplantations made in the Brno Centre of Cardiovascular and Transplantation Surgery. One-year survival in the group of patients is 80%, three-year survival 69%. The authors discuss indications and contraindications of cardiac transplantations, necessary preoperative and postoperative examinations, they follow-up the most serious complications during the posttransplantation period. They mention therapeutic possibilities and outline briefly the perspectives of care of patients after cardiac transplantation. Despite the number of problems encountered transplantation of the heart is for indicated patients with cardiac failure a unique chance to improve the prognosis of survival and the quality of life.


Subject(s)
Heart Transplantation , Adult , Contraindications , Heart Transplantation/adverse effects , Heart Transplantation/mortality , Humans , Postoperative Care , Postoperative Complications , Preoperative Care , Survival Rate
5.
Cas Lek Cesk ; 131(10): 315-6, 1992 May 25.
Article in Czech | MEDLINE | ID: mdl-1638596

ABSTRACT

The authors give an account of a bioptically revealed adenocarcinoma of the large bowel which was combined with a carcinoid. The carcinoid constituent of the tumor had massive metastases as revealed by the postmortem examination.


Subject(s)
Adenocarcinoma/pathology , Carcinoid Tumor/pathology , Colonic Neoplasms/pathology , Neoplasms, Multiple Primary/pathology , Aged , Humans , Male
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