ep? ae S te been ‘a ehad ~ Stores. *. id high titres of 'g frican cronesians comerous megakaryocytes in the bone-marrow and some in Sveipheral blood, the possibility that the patient might have a ys. was raised. Ses been obtained. ‘three. whe Ouchterl ony “ef No convincing evidence that this is so -@yhout transfusion the patient’s hemoglobin continued to 2, and in February, 1958, he was admitted to the Clinical -_. €$ Containing Se .-< ‘f sodium Dhos Rhea ni a: m_ ethylene ee d:, sheet Wirth a dic, six we ‘Well of the Same ts. and Peripher al y- Center of the National Institutes of Health. During his time in “Were plac ed in Fig. i . Fig. 2 % I—Fhe precipitin reaction, seen in an agar-gel* Ouchterlony by Oblique lum, _ plate, between the serum of the patient in the centre well and by the mictom esdey idegger (1955 Lusty ° 3-qeene, but not all, members of a panel of sera from normal 3 pervons. Unstained piate after 24 hours. agar made up .. _ Me 3—The precipitin reaction between the serum of the patient in ody (or antigen wr: “€acting for 18 ha. -. Were visible. Jz, _,¥ te centre well against some, but not all, members of a panel of + The cwo degrees of positive reaction are shown: nos. 1; 3, and 5 + ga 2+ ceactors and no. 2 is a weak + reactor. vhich the Plates Wee- Connaught Lahn ‘droxymeth! -2n-:+,. Secpitel the patient had persistent anzmia withoutreticulocywas. The leucocyte count varied from 10,000 to 15,000 and the platelet count was consistently above 500,000 per c. mm. ‘The differential count showed predominantly neutrophils with tciztive increase of immature forms. Bone X-rays showed no W906 80. 1507. aste (Smithies po<Was Carried outs - he components w:: iS componensys we ities, Refractory anemia of unknown cause was Gagnosed. The patient was given cells from 4 units of blood, Wiech raised his hemoglobin to 11 g. per 100 ml., and was ee ‘peration of 7S as: Oacharged. © density-cradie: ~ When the patient first received blood-transfusions in 1958 |» Seer were well tolerated. Since that time he has received 47 7) using 40, 30, > wea of blood. The patient is of blood group AB, Rh+, and bas never been evidence of red-cell incompatibility with Mf Bozicevich cpa ", emer bloods. In the spring of 1960 the patient began to have IE aS describe tia) 4 . “Sxnrfusion reactions, Within about 1 hour of some, but notall, famtfusions there was a fever, rising to 38-39°C (100-4— * $022°F) accompanied by headache and muscle pain, but no symptoms. The fever and symptomspersisted for about of a patient wh. t long and varie A sc.end positive subjects had rheumatoid factor, as determined by bentonite-flocculation and latex-fixation tests. Some panel subjects with rheumatoid factor pave reactions with the antiserum, but others did not. A second sample of serum from the patient, drawn 3 weeks after thefirst, ata we andere Fig. 4—Agar-gel antibody. . -% i immunocelectrophoresis . oo to _ characterise the The serum of the patient was placed in the circular wells and submitted to electrophoresis, and a strongly positive serum concentrated three times was placed in the rectangular wells. A broad precipitin line (arrow) is seen in the position of the y-globulin. time when the patient had not received any medication since discharge, gave identical reactions, as did a third sample drawn 1 month Jater, and a fourth 3 monthslater. Support for the interpretation that the precipitin in the serum of this patient is an antibody came from experi- ments showing that the active protein is a 7S-y-globulin. the a-globulin antigen and Ag(a~) for sera that do not contain the antigen. The precipitin in the serum of the patient thus has the specificity anti-Ag(a-+-), buminuria whit nths. unlike the diffuse reaction which slowly develops between © rheumatoid factor and y-globulin in some subjects (Franklin 1960a). Rheumatoid-factor reaction was excluded because neither the patient nor most of the We propose the designation Ag(a+) for sera containing Ll retired Cecwrtiy Budapest. he het d paralysis of the Precipitation Reactions The serum of the patient gave well-defined precipitation reactions in agar with some, but not all, panel sera (fig. 1). The reaction was clearly visible at 18 hours and was intense after 2 days. The precipitation line was convex toward the centre (precipitin) well, indicating that the antigen is of relatively high molecular weight (Korn- (fig. 1). The precipitation lines were clear-cut and quite d at 18 houn ~ ally Visible}, 25 han ‘ RA-tess* i 5 hours, and then slowly subsided. The patient had been discharged from hospital before the precipitin in his blood was discovered, so that it has not yet been possible to ascertain whether only bloods containing the precipitating antigen give transfusion reactions. gold et al. 1959). Two intensities of reaction were defin- tse ral wells. Th e pln 2rve nd 635 able in most groups of sera studied (fig. 2). Some sera lacked detectable antigen, the precipitation lines extending into the corresponding wells without deviation * 88ar cores frovith small volu - ta ma - tebe THE LANCET ORIGINAL ARTICLES Ee ipved for a month and then ceased, and with the help of sfusions the hamoglobin was raised to 14 g. per ml. Because of the high white-cell count and the presence debi years. S$ : € Sera Were «. 025, 1961 Characterisation of Antigen E.. A panel serum giving a strongly positive precipitation line in the gel-diffusion test was concentrated three times by ultrafiltration and used for immunoelectrophoresis. Waist, Weeknat in the legs. Sod spinal turd s.r: per ml. Pycya2ain remiss The antibody well wasfilled with serum from the patient. A well-defined precipitation line developed between the Iceration daticg 1938 and lhe but svmpis: antigen is a high-molecular-weight x,-component. “he patiens war he was repericevels, and tus’ ION suggesize The eesics %, region and the antibody (fig. 3). This result, along with that in the Ouchterlony plates, suggested that the i. t~Agar-gel immunoelectrophoresis to characterise antigen. tenes beulti a strongly Positive reactor was concentrated three ef the Patient wasn and submitted to electrophoresis. The serum =ion of the as placed in the rectangular antibody well. The Sobulin Abovern line (arrow) suggests that the antigen is an . Ove, unstained precipitin lines; below, protein stain. en re The only a,-macroglobulin so far defined is the 19S-«, component described by Brown et aj. (1954) and Wallenius et al. (1957), which appears to be equivalent to the’ slow-x, component found on starch-gel electrophoresis (Poulik and Smithies 1958). Purified «,-macroglobulin (Behringwerke, Marburg) in a wide range of concentrations failed to give precipitation with the patient’s serum. m2

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