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Synthesis of abnormal immunoglobulins in lymphoplasmacytic disorders with visceral light chain deposition
purpua Spontaneous agents
recovery Treatment
failures
disorders
immunogiobuiin
Peiiosis hepatitis MydoItW synthesis
Kinetics
aggregation
immunogiobuiin
G
Storage pool deficiency
Acquired abnormalities of platelet aggregation have been reported with increasing frequency. Skafied here were five patients with impaired platelet aggregation responses to collagen, epinephrine and ADP: in all cases, increased levels of platelet-associated immunoglobulin G (IgG) were found. In all five patients contents of substances stored in plateletdense ganules (ATP. ADP. serotonin and calcium) were diminished. The content of the a-granule substance &thromboglobulin was also decreased in most cases, whereas the levels of two secretable add hydrolase enzymes @+cuonidase and &N-acetyl glucosaminidase) were normal. These findings are similar to those observed in subtypes of congenital storage pool deficiency. However, in contrast to the congenital disorder, a membrane-bound (nonsecretable) acid phosphatase was also decreased in the patients with acquired storage pool deficiency. These findings suggest that irrpaired platelet aggegation ~1 an acquired basis may. in some patients. be due to immune platelet damage resulting in a distinctive type of platelet storage pool deficiency.
Weiss HJ. Rosove MH. Lages EA. Kaplan KL: Acquired storage pool deficiency with increased platelet associated IgG. Report of five cases. Am J Med 1980; 69: 71 l717.
Platelet
Neutrophil kinetics were measued in 19 patients with Felty’s syndrome. In all but two patients, neutropenia was associated with no increase in granulocytopoiesis, despite evidence for accelerated marrow release and disappearance of mature neutrophils. Antineutrophil antibodies were detected in seven of nine patients. Skin exudate cellularii was greaterin patients asymptcmatic from infection. Sustained naulrophll increments were observed in six of 10 patients after splenectcmy, but in no patient did neutrophil kinetics return to normal. Five patients with in&equate ~locytc@esis were treated with lithium without effect. Althwgh multiple factors may be involved in the pathogenasis of neutropenia in Felty’s syndrome, granulocyte kinetics most consistently demonstrate inadequate granulocytopoiesis. The severity of this impairment may be helpful in predicting response to splectomy.
P: Neutrophil kinetics in Felty’s syn-
Feity’s syndrome
Joyce RA. Boggs DFI, Chervenick PA, Lalezari drome. Am J Mad 1980: 69: 695-702.
Neutropenia
Neutrophiis
Conlrnued on page A3(l
Three @ants f-d tissua depoaks raaclive with anti-K seruns. They had progessive renal failue with nephrotic syndrome and one had a peliosis hepatis. A monoclonal lymphoplasmacytic diswder (myeloma. pleomorphic lyrnphoid malignancy or monoclonal plasmacytosls wlthout overt myeloma) was found in all patients. Biosynthesis experiments showed the production of light chain with unusual properties (abar~ant size. tendency to polymerize. possibly glycosylatbn). A causal relationship between synthesis of abnormal immunoglobulins and tissue deposition is suggested.
Preud’homme JL, Morel-MaroQar L. Brouat JC. Cerf M. Mignon F. Guglielmi P. Seligmann M: Synthesis of abnormal immunoglobulins in lymphoplasmacylic disorders with visceral light chain deposition. Am J Med 1980; 69:703-710.
Lymphopiasrnacytk
Nephrotic syndrome
Df 38 adult patients wim idiopathic thrombocytopenic purpura followed an average of rno+e than 12 years, 15 suffered splenectomy failure or postsplenectomy recwTence of thrombocytopenia. Nine of the 15 also received immunosuppressive agents, and four of the nine failed such therapy. h ei$t of these 15 treatment failues nzmal or safe platelet counts were achieved in a subsequent three to 12 year period during which they received no therapy. The frequency of spontanews recovery of satisfactory pbtelet levels In adults wlth MlopatMc thrcxnbocytopenic purpura who were treatment failures may have negative implications for very vigorous or long-standing immunosuppressive therapeutic attempts in certain cases.
Picoui VJ. Roeske WR. Creger WP: Fate of therapy failures in adult idiopathic throm bocytopenic purpura. Am J Med 1980: 69:690-694.
inwmmosuppres.sive
idiopathic thrombocytopenic
Spienectomy
recovery Treatment
failures
disorders
immunogiobuiin
Peiiosis hepatitis MydoItW synthesis
Kinetics
aggregation
immunogiobuiin
G
Storage pool deficiency
Acquired abnormalities of platelet aggregation have been reported with increasing frequency. Skafied here were five patients with impaired platelet aggregation responses to collagen, epinephrine and ADP: in all cases, increased levels of platelet-associated immunoglobulin G (IgG) were found. In all five patients contents of substances stored in plateletdense ganules (ATP. ADP. serotonin and calcium) were diminished. The content of the a-granule substance &thromboglobulin was also decreased in most cases, whereas the levels of two secretable add hydrolase enzymes @+cuonidase and &N-acetyl glucosaminidase) were normal. These findings are similar to those observed in subtypes of congenital storage pool deficiency. However, in contrast to the congenital disorder, a membrane-bound (nonsecretable) acid phosphatase was also decreased in the patients with acquired storage pool deficiency. These findings suggest that irrpaired platelet aggegation ~1 an acquired basis may. in some patients. be due to immune platelet damage resulting in a distinctive type of platelet storage pool deficiency.
Weiss HJ. Rosove MH. Lages EA. Kaplan KL: Acquired storage pool deficiency with increased platelet associated IgG. Report of five cases. Am J Med 1980; 69: 71 l717.
Platelet
Neutrophil kinetics were measued in 19 patients with Felty’s syndrome. In all but two patients, neutropenia was associated with no increase in granulocytopoiesis, despite evidence for accelerated marrow release and disappearance of mature neutrophils. Antineutrophil antibodies were detected in seven of nine patients. Skin exudate cellularii was greaterin patients asymptcmatic from infection. Sustained naulrophll increments were observed in six of 10 patients after splenectcmy, but in no patient did neutrophil kinetics return to normal. Five patients with in&equate ~locytc@esis were treated with lithium without effect. Althwgh multiple factors may be involved in the pathogenasis of neutropenia in Felty’s syndrome, granulocyte kinetics most consistently demonstrate inadequate granulocytopoiesis. The severity of this impairment may be helpful in predicting response to splectomy.
P: Neutrophil kinetics in Felty’s syn-
Feity’s syndrome
Joyce RA. Boggs DFI, Chervenick PA, Lalezari drome. Am J Mad 1980: 69: 695-702.
Neutropenia
Neutrophiis
Conlrnued on page A3(l
Three @ants f-d tissua depoaks raaclive with anti-K seruns. They had progessive renal failue with nephrotic syndrome and one had a peliosis hepatis. A monoclonal lymphoplasmacytic diswder (myeloma. pleomorphic lyrnphoid malignancy or monoclonal plasmacytosls wlthout overt myeloma) was found in all patients. Biosynthesis experiments showed the production of light chain with unusual properties (abar~ant size. tendency to polymerize. possibly glycosylatbn). A causal relationship between synthesis of abnormal immunoglobulins and tissue deposition is suggested.
Preud’homme JL, Morel-MaroQar L. Brouat JC. Cerf M. Mignon F. Guglielmi P. Seligmann M: Synthesis of abnormal immunoglobulins in lymphoplasmacylic disorders with visceral light chain deposition. Am J Med 1980; 69:703-710.
Lymphopiasrnacytk
Nephrotic syndrome
Df 38 adult patients wim idiopathic thrombocytopenic purpura followed an average of rno+e than 12 years, 15 suffered splenectomy failure or postsplenectomy recwTence of thrombocytopenia. Nine of the 15 also received immunosuppressive agents, and four of the nine failed such therapy. h ei$t of these 15 treatment failues nzmal or safe platelet counts were achieved in a subsequent three to 12 year period during which they received no therapy. The frequency of spontanews recovery of satisfactory pbtelet levels In adults wlth MlopatMc thrcxnbocytopenic purpura who were treatment failures may have negative implications for very vigorous or long-standing immunosuppressive therapeutic attempts in certain cases.
Picoui VJ. Roeske WR. Creger WP: Fate of therapy failures in adult idiopathic throm bocytopenic purpura. Am J Med 1980: 69:690-694.
inwmmosuppres.sive
idiopathic thrombocytopenic
Spienectomy