[Diagnosis and therapy of pneumocystis carinii pneumonia in a patient with AIDS]. / Diagnostik und Therapie einer Pneumocystis-carinii-Pneumonie bei einem Patienten mit AIDS.

The clinical, immunological and histological findings in a patient suffering the acquired immunodeficiency syndrome and Pneumocystis carinii pneumonia are discussed. At the time of diagnosis HTLV III antibodies were not demonstrable. Treatment with trimethoprim sulfamethoxazole achieved a remission lasting 7 months so far. HTLV III antibodies became demonstrable after 5 months; the markedly decreased Helper/Suppressor ratio of 0.30 remained unchanged.

[The diagnosis of IgD-Myelomatosis (author's transl)]. / Zur Diagnose des IgD-Plasmozytoms

Two patients with IgD-lambda myelomatosis are presented and the differential diagnosis is discussed. Typical features of this disease are the high incidence of Bence-Jones proteinuria, osteolytic lesions, amyloidosis and the predominance of male patients. Furthermore, an augmentation of serum IgD level to 165 mg% was observed in a 22-year-old female patient with presumed Coxsackie myocarditis. The theories in regard to IgD function are discussed.

[Immunology of the Goodpasture's syndrome]. / Zur Immunologie des Goodpasture-Syndroms

About 5% of all human glomerulonephritides are induced by basal membrane antibodies. Immunohistologically they are characterized by a linear fluorescence along the basal membrane of the glomerulum, when cuts of the kidneys of bioptic material are incubated with fluorescent anti-human-IgG-serum. Basal membrane antibodies circulating in the serum as well as antibodies eluted from renal homogenates are nephritogenic. Also recidivations of nephritis on renal homografts confirm the pathogenic significance. Basal membrane antibodies of patients with rapidly progressing glomerulonephritis seem to differ only by a non-existing cross-reaction to the antigen of the basal membrane of the lungs from Goodpasture basal membrane antibodies. Only a glomerulonephritis with pulmonary haemorrhage induced by basal membrane antibodies should be called Goodpasture syndrome.

Symptoms and immunology of the Henoch-Schönlein-syndrome.

The Henoch-Schönlein-syndrome, or anaphylactoid purpura, is usually a childhood disease. The progess of this syndrome in adults was observed in 4 cases at our clinic. An elevated IgA level in the serum with a normal complement value seems to be of immunological interest. By immunohistological methods fibrin and IgA could be demonstrated in skin and kidney biopsies; in glomerulae C3c was observed, occasionally accompanied by Properdin-factor-B. These findings differentiate this disease from other allergic superficial vasculitids by the detection of IgG and complement; while at the same time some similarities are indicated to the so called "Berger Nephritis" with comparable kidney findings.

[Essential mixed cryoglobulinemia with kidney involvement]. / Essentielle gemischte Kryoglobulinämie mit Nierenbeteiligung

Essential mixed cryoglobulin associated with rapidly progressive glomerulonephritis has been observed in a 64-year-old patient. The cryoglobulin consisted of monoclonal IgM-kappa and polyclonal IgG and displayed rheumatoid-factor activity. The antinuclear antibody test was negative. In the immunofluorescent method renal biopsy showed positive staining for IgG; IgM and the early and late complement components. The rheumatoid factor activity of the renal IgM deposits was demonstrated by binding of fluorescent aggregated IgG. There is thus evidence that the vasculitis observed in essential mixed cryoglobulin is caused by IgM-IgG complexes. The differentiation of essential mixed cryoglobulin from lupus nephritis with cryoglobulins is discussed.

[Rare abdominal manifestations of tuberculosis: isolated splenic tuberculosis and peritoneal tuberculosis]. / Seltene abdominale Tuberkulose-manifestationen: isolierte Milztuberkulose und Peritonealtuberkulose.

Two patients with rare localizations of extrapulmonary tuberculosis are presented. In a 55-year-old man admitted with septic fever and a low neutrophil count, ultrasonography of the spleen revealed splenomegaly with numerous hypodense foci. Fine needle biopsy of one of the foci disclosed tuberculosis. Persistent splenogenic neutropenia and thrombocytopenia, despite tuberculostatic therapy, required splenectomy which confirmed the initial diagnosis. At laparotomy no other abdominal foci of tuberculosis were found. In a second 57-year-old man admitted for ascites and weight loss, a biopsy specimen of the liver showed normal liver parenchyma but, by chance, peritoneal tuberculosis. Tuberculostatic therapy brought about cure.

[Goodpasture's syndrome (author's transl)]. / Klinik und immunologie des goodpasture-syndroms

Goodpasture's syndrome occurred in six patients (four men and two women) aged 18-32 years. Haemoptysis, anaemia and abnormal urinary findings with erythrocyturia were the first signs of the disease in five instances, in one the renal signs predominated. In five patients the disease took a malignant course with uraemia developing within six weeks. One patient is in a spontaneous remission eleven months after the onset of the disease. Three patients died of recurrent haemoptysis (non-nephrectomised), two nephrectomised patients are still alive half a year and three and a half years later, respectively. A typical linear IgG-, beta1A, alpha2D and beta1E deposition along the glomerular capillaries was demonstrated immunohistologically in all subjects. Of three lung biopsies one also had typical IgG and beta1A-positive linear fluorescence along the alveolar basal membrane. Immunoglobulins with basal membrane antibody activity were eluted from renal homogenates in two patients, basal membrane antibody activity in serum was demonstrated only in one of three patients.

[Wegener's granulomatosis as a rare cause for kidney failure]. / Wegenersche Granulomatose als seltene Ursache einer Niereninsuffizienz.

Course and treatment of a case of Wegener's granulomatosis are reported. A 39-year-old man admitted to the hospital because of abnormal densities demonstrated by the X-ray examination of the chest. The patient developed a rapidly progressive renal failure with renal insufficiency. Diagnosis was obtained by biopsys from the upper respiratory tract and from the kidney. During hemodialysis and immunosuppressive therapy renal function improved and hemodialysis could be stopped. The patient was discharged from the hospital and kept on immunosuppressive therapy. The patient omitted the medication by himself and renal insufficiency reappeared. The disease was progressive and the patient died because of intestinal bleeding. Apart from the description of the symptoms of this rare disease, problems of diagnosis and treatment are discussed.

Surface immunoglobulins and receptor sites for aggregated IgG on leukemic reticuloendotheliosis cells.

On blood lymph nodes and spleen cells of 4 patients with leukemic reticuloendotheliosis (LR) the binding of 125-J-labelled IgG aggregates as well as binding of 125J-labeled anti immunoglobulin sera was studied. Moreover the capacity to form spontaneous rosettes with sheep red blood cells to bind immune complement complexes as well as the proliferative response in the presence of PHA were investigated. On the surface of hairy cells a variety of immunoglobulins were demonstrable. IgG of both types was found to be the predominant surface bound immunoglobulin and evidence was obtained, that these antibodies were adsorbed from the serum via Fc-receptors. In all LR-cases the percentages of T-cell rosettes as well as the PHA responses were decreased when compared with the normal controls. The numbers of immune complement complex binding cells were in the range of the normal controls. Results are discussed with respect to the origin of hairy cells in LR.

[Plasmocytoma, alkylating agents, and acute myeloid leukemia (author's transl)]. / Plasmozytom, Alkylantien und akute myelogene Leukämien

Two cases of the development of acute myeloid leukemia (AML) after treatment with alkylating agents are reported. In Case 1, melphalan and then cyclophosphamide had been given for multiple myeloma. 46 months after onset of cytostatic treatment AML occurred, as confirmed cytochemically and by qualitative determination of urinary lysozyme. In Case 2, cyclophosphamide had been given for rheumatoid arthritis. After a latency of 34 months 'smouldering leukaemia' developed with an atypical monocytic leukaemic cell population. In a third case, multiple myeloma and monocytic leukaemia developed synchronously. The causative role of melphalan and cyclophosphamide in the development of AML seems securely established. Despite the risk of alkylating agents in the treatment of multiple myeloma or Hodgkin's disease causing AML, they should not be replaced, as other drugs have been shown to be less beneficial. On the other hand, alkylating agents should be used with great caution in the treatment of non-malignant diseases.

[Non-secreting myeloma. Case report with immunohistologic and electron microscopic studies]. / Das nicht-sezernierende Myelom. Ein Fallbericht mit immunhistologischen und elektronenmikroskopischen Untersuchungen.

Immunological, immunofluorescence and electromicroscopic studies were performed in a case of atypical myeloma. The 77-year-old patient presented with skeletal pain, multiple osteolytic lesions and bone marrow infiltration by atypical plasma cells. Monoclonal light chains kappa were confined to the plasma cells, as shown by immunofluorescence. No monoclonal immunoglobulin or fragments were detected in plasma or concentrated urine, even by highly sensitive immunological methods. The concentration of the immunoglobulins G, A and M in the plasma was markedly reduced. The plasma cells contained very little sarcoplasmatic reticulum. The simultaneous occurrence of monoclonal light chains kappa in the plasma cells and the absence of monoclonal immunoglobulins or fragments in plasma and urine suggest a non-secretory myeloma.