scholarly journals Hemorrhagic plaques in the oral cavity: A clue to diagnosing thrombocytopenia

2021 ◽  
Vol 12 (2) ◽  
pp. 166-166
Author(s):  
Aswathi Raj ◽  
Amina Asfiya ◽  
Malcolm Pinto ◽  
Manjunath Shenoy M ◽  
Spandana P Hegde ◽  
...  
Keyword(s):  
Oral Cavity ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Oral Manifestations ◽  
Platelet Destruction ◽  
Thrombocytopenic Purpura ◽  
Platelet Production ◽  
Gingival Bleeding ◽  
Clinical Presentations ◽  
Wide Range ◽  
Hemorrhagic Bullae

Idiopathic thrombocytopenic purpura is a disorder with a myriad of possible clinical presentations. The mechanism of thrombocytopenia involves both increased platelet destruction and impaired platelet production. The patient can manifest a wide range of symptoms: from asymptomatic or minimal gingival bleeding to profuse bleeding from any site. The disease may first present itself to the dermatologist in cutaneous findings such as petechiae, purpura, and mucosal manifestations in the form of gingival bleeding and hemorrhagic bullae. The diagnosis of idiopathic thrombocytopenic purpura is mostly done by exclusion. In this report, we present two cases with characteristic oral manifestations, who were diagnosed, on investigation, with idiopathic thrombocytopenic purpura. The patients were successfully treated with immunosuppressive therapy. The report aims to raise awareness that would help in enabling prompt referral to the appropriate specialty, especially because of the rarity of this presentation.

Platelet Production Rate Predicts the Response to Prednisone Therapy in Patients with Idiopathic Thrombocytopenic Purpura.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1325-1325
Author(s):  
Ewout Houwerzijl ◽  
Henk Louwes ◽  
Wim Sluiter ◽  
Jan Smit ◽  
Edo Vellenga ◽  
...  
Keyword(s):  
Production Rate ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Therapeutic Strategy ◽  
Patient Characteristics ◽  
Platelet Destruction ◽  
Thrombocytopenic Purpura ◽  
Platelet Production ◽  
Prednisone Therapy ◽  
The Mean

Abstract The thrombocytopenia in ITP is predominantly caused by autoantibodies that recognize antigens on platelets and megakaryocytes resulting in accelerated platelet destruction and a decreased platelet production rate (PPR). ITP patients with a predominantly suppressed PPR might respond differently to therapy than patients with predominantly peripheral platelet destruction. Tests and/or patient characteristics that can make a distinction between a reduced platelet half life and a reduced PPR could therefore influence diagnostic and therapeutic strategy in ITP. Therefore, in the present study the predictive value of clinical and platelet kinetic parameter for treatment outcome in idiopathic thrombocytopenic purpura (ITP) was investigated. Seventy-five patients with severe ITP (platelets ≤20 × 109/L) were studied. Median age was 46 (range 16–89) years and 34 (45%) patients were males. Median platelet count was 8 (1–20) × 109/L. The mean platelet life was 1 (0.1–6.5) days, and the PPR 160 (2–4670) × 109/day (normal 223 (100–355) × 109/day, p = 0.7). PPR was decreased (<100 × 109/day), normal (100–355 × 109/day) and increased (>355 × 109/day) in 33%, 48%, and 19% of the patients, respectively. All patients started with prednisone at diagnosis (1 mg/kg/day). Initial complete and partial response (CR/PR) was 84% and a durable CR/PR (defined as CR/PR for ≥6 months without treatment) was attained in 44% of the patients. Apart from a higher proportion of patients with a decreased PPR in the group that responded to prednisone therapy (p=0.03), there were no significant differences regarding clinical and platelet kinetic parameters between responders and nonresponders. A durable CR/PR was noticed in 64% of the patients with a decreased PPR (median follow-up of 81 months (18–92)), compared to 34% of the patients with normal or increased PPR (median follow-up 141 (10–284) months (HR: 0.47 [95% CI (0.24–0.92)], p = 0.03). Splenectomy was performed in 32% of the patients with decreased PPR and in 62% of patients with normal or increased PPR (p = 0.03). In addition, patients with a decreased PPR showed significantly less splenic sequestration and a significantly longer mean platelet life than patients with a normal or increased PPR, which underscores that in these patients peripheral destruction of platelets contributes relatively less to thrombocytopenia than suppressed platelet production. Thirty-nine of 42 nonresponders to prednisone underwent splenectomy. Durable CR/PR postsplenectomy was reached in 74%. There were no significant differences with regard to patient characteristics between responders and nonresponders to splenectomy. In conclusion, ITP patients with suppressed PPR have a significant higher durable CR/PR rate to prednisone therapy and are less frequently exposed to splenectomy, than those with a normal or increased PPR.

Idiopathic thrombocytopenic purpura: Current concepts in pathophysiology and management

2008 ◽  
Vol 99 (01) ◽  
pp. 4-13 ◽  
Author(s):  
Maria Laura Evangelista ◽  
Elisa Stipa ◽  
Francesco Buccisano ◽  
Adriano Venditti ◽  
Sergio Amadori ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Immune Modulation ◽  
Cellular Mechanisms ◽  
Platelet Destruction ◽  
Thrombocytopenic Purpura ◽  
Platelet Production ◽  
Structural Resemblance ◽  
Thrombopoietin Receptor ◽  
Low Platelet Count

SummaryIdiopathic thrombocytopenic purpura (ITP) is characterized by a low platelet count, which is the result of both increased platelet destruction and insufficient platelet production. Although the development of autoantibodies against platelet glycoproteins remains central in the pathophysiology of ITP, several abnormalities involving the cellular mechanisms of immune modulation have been identified. Conventional treatments for ITP aim at reducing platelet destruction, either by immunosuppression or splenectomy. Two new thrombopoietic agents, AMG 531 and eltrombopag, have been used in clinical trials to stimulate platelet production in ITP patients not responsive to standard treatments. These new molecules bear no structural resemblance to thrombopoietin, but still bind and activate the thrombopoietin receptor. This review will focus on the pathophysiology and treatment of ITP in adults, highlighting recent advances in both fields.

scholarly journals Dental considerations on the management of Idiopathic Thrombocytopenic Purpura in children: case report

2015 ◽  
Vol 63 (4) ◽  
pp. 472-476
Author(s):  
Viviane Ferreira ROSSIER ◽  
Stella Maria Coda Pinto Alves Campos VIEIRA ◽  
Ana Lídia CIAMPONI ◽  
Renata de Oliveira GUARÉ
Keyword(s):  
Idiopathic Thrombocytopenic Purpura ◽  
Blood Platelets ◽  
Clinical Signs ◽  
Oral Manifestations ◽  
Hematological Disease ◽  
Thrombocytopenic Purpura ◽  
Adequate Treatment ◽  
Gingival Bleeding ◽  
Dental Office ◽  
Acute Itp

Idiopathic Thrombocytopenic Purpura (ITP) is a hematological disease characterized by decreased number of blood platelets. Clinically, children with ITP may present petechiae, ecchymoses, haematuria, epistaxis and occasionally hemorrhage. Oral manifestations include spontaneous gingival bleeding, petechiae or hematomas of the mucosa, palate and tongue. It is important for dentists to be aware of ITP in order to properly recognize this condition and offer the adequate treatment to the patient. The aim of this report was, therefore, to relate the case of a 4-year-old patient with acute ITP, to review its main clinical signs in children and describe the management of these patients at the dental office.

scholarly journals Review article: THE ROLE OF ELTROMBOPAG AND ROMIPLOSTIM AS THE THROMBOPOIETIN RECEPTOR AGONIST (TPO-RA) IN TREATMENT OF IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP): WHAT IS TPO-RA, WHEN TPO-RA IS USED AND HOW TO TAKE TPO-RA?

2016 ◽  
Vol 51 (3) ◽  
pp. 203 ◽  
Author(s):  
Jefri Pratama Susanto
Keyword(s):  
Idiopathic Thrombocytopenic Purpura ◽  
Receptor Agonist ◽  
Dose Adjustment ◽  
Review Article ◽  
Platelet Destruction ◽  
Thrombocytopenic Purpura ◽  
Platelet Production ◽  
Thrombopoietin Receptor ◽  
Thrombopoietin Receptor Agonist

Idiopathic thrombocytopenic purpura is a autoimmune disease characterized thrombocytopenia casued by excessive platelet destruction. However, both platelet destruction and reduced thrombopoietin level are occurred in some cases. Therefore, new management of ITP is emerged which target is to increase platelet production rate via eltrombopag or romiplostim as the thrombopoietin receptor agonist (TPO-RA). Eltrombopag is given orally while romiplostim is given subcutaneously or intravenously and dose adjustment is depend on platelet count. Both eltrombopag and romiplostim is indicated in minimal response glucocorticoid or intravenous immunoglobulin or splenetomy treatment.

PAICA: A Method for Characterizing Platelet-Associated Antibodies - Its Application to the Study of Idiopathic Thrombocytopenic Purpura and to the Detection of Platelet-bound c7E3

1996 ◽  
Vol 76 (06) ◽  
pp. 1020-1029 ◽  
Author(s):  
Laurent Macchi ◽  
Gisèle Clofent-Sanchez ◽  
Gérald Marit ◽  
Claude Bihour ◽  
Catherine Durrieu-Jais ◽  
...  
Keyword(s):  
Monoclonal Antibody ◽  
Flow Cytometry ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Antithrombotic Therapy ◽  
Membrane Glycoproteins ◽  
Serum Antibodies ◽  
Platelet Destruction ◽  
Thrombocytopenic Purpura ◽  
Capture Assay ◽  
Specific Membrane

SummaryIn idiopathic thrombocytopenic purpura (ITP), autoantibodies reacting with antigens on the platelet membrane bring about accelerated platelet destruction. We now report PAICA (“Platelet-Associated IgG Characterization Assay”), a method for detecting autoantibodies bound to specific membrane glycoproteins in total platelet lysates. This monoclonal antibody (MAb) capture assay takes into account the fact that antibodies on circulating platelets may be translocated to internal pools as well as being on the surface. A total of twenty ITP patients were examined by PAICA, and the results compared with those obtained by measuring (i) serum antibodies bound to paraformaldehyde-fixed control platelets by ELISA, (ii) IgG bound to the surface of the patient’s own platelets by flow cytometry (PSIgG), (iii) total platelet-associated IgG (PAIgG) by ELISA and (iv) serum antibodies reacting with control platelets by MAIPA (“Monoclonal Antibody-specific Immobilization of Platelet Antigens”). Of twelve patients with elevated PAIgG, nine had increased PSIgG yet eleven reacted positively in PAICA. Of these, eight possessed antibodies directed against GP Ilb-IIIa, two against GP Ib-IX and one patient possessed antibodies directed against GP Ilb-IIIa and GP Ia-IIa respectively. Only seven of the patients possessed serum antibodies detectable by MAIPA. PAICA was also able to detect platelet-associated c7E3 (the chimeric form of Fab fragments of the MAb 7E3) following its infusion during antithrombotic therapy, when it proved more sensitive over a seven-day period than a MAIPA assay adapted for assessing surface-bound antibody. We propose that PAICA provides added sensitivity to the detection of platelet-associated antibodies in immune thrombocytopenias or following therapy with humanized MAbs.

scholarly journals Spectrum of Ig classes, specificities, and titers of serum antiglycoproteins in chronic idiopathic thrombocytopenic purpura

Blood ◽  
1994 ◽  
Vol 83 (4) ◽  
pp. 1024-1032 ◽  
Author(s):  
R He ◽  
DM Reid ◽  
CE Jones ◽  
NR Shulman
Keyword(s):  
Idiopathic Thrombocytopenic Purpura ◽  
Cytoplasmic Domain ◽  
Platelet Glycoprotein ◽  
Chronic Idiopathic Thrombocytopenic Purpura ◽  
Platelet Destruction ◽  
Thrombocytopenic Purpura ◽  
Secondary Phenomenon ◽  
Almost All ◽  
Posttransfusion Purpura ◽  
Detectable Serum

Abstract The characteristic decreased recovery and survival of transfused platelets in nonalloimmunized patients with idiopathic thrombocytopenic purpura (ITP) suggest that plasma antiplatelet autoantibodies (autoAbs) are present in almost all cases. Studies emphasizing reactions of IgG autoAbs with platelet glycoprotein (GP) IIb/IIIa indicate that less than 50% of ITP patients have detectable serum Abs, and that many of these Abs may not be pathogenic because they are directed against epitopes in the cytoplasmic domain of GPIIIa (Fujisawa et al, Blood 77:2207, 1991 and 79:1441, 1992). We evaluated the contribution of Ig classes other than IgG to the overall incidence of serum Abs in 47 patients with chronic ITP and the frequency of reactions with GPs IIb/IIIa, Ib/IX, IV, and Ia/IIa. Abs were further characterized by their reactions with cytosolic or exosolic GP epitopes and their titers and apparent affinities. Using immunobead techniques we found (1) anti- GPs in 85% of sera; (2) IgA and IgG Abs each in 68%, together in 51%; (3) IgM agglutinins in 15%, always with another Ab class; (4) GP Ib/IX, IIb/IIIa, IV, and Ia/IIa targets in 83%, 81%, 38%, and 28% of cases, respectively; (5) 93% of positive sera reactive with more than one GP; but GP IV or Ia/IIa never the sole target; (6) Abs against cytosolic epitopes on one or more of GPs IIIa, Ib alpha, and IIb beta in 66% of sera, always accompanied by Abs against exosolic epitopes of the same or a different GP; (7) autoAbs against cytosolic GP epitopes in 38% of 16 patients recovered from posttransfusion purpura and drug purpura; and (8) evidence that serum ITP Abs, often high-titered, saturate platelets less than alloAbs against the same GPs. Whereas Abs against external GP epitopes are a distinctive marker for ITP in 80% of patients, Abs against internal GP epitopes are likely a secondary phenomenon of platelet destruction and not pathogenic. Anti-GPs against exosolic epitopes were also found in eluates of patients platelets', suggesting that they have pathogenic significance.

scholarly journals Evaluation by quantitative acid elution and radioimmunoassay of multiple classes of immunoglobulins and serum albumin associated with platelets in idiopathic thrombocytopenic purpura

Blood ◽  
1986 ◽  
Vol 67 (4) ◽  
pp. 1126-1131 ◽  
Author(s):  
AJ Hotchkiss ◽  
CA Leissinger ◽  
ME Smith ◽  
JV Jordan ◽  
CA Kautz ◽  
...  
Keyword(s):  
Serum Albumin ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Solid Phase ◽  
Similar Group ◽  
Thrombocytopenic Purpura ◽  
Large Numbers ◽  
Wide Range ◽  
Platelet Concentration ◽  
Associated Proteins ◽  
Solid Phase Radioimmunoassay

Abstract Immunoglobulins (Igs) and serum albumin were eluted from normal platelets and platelets from patients with idiopathic thrombocytopenic purpura (ITP) with a quantitative acid elution procedure followed by solid-phase radioimmunoassay (SPRIA). Acid elution was shown to release a reproducible fraction of platelet-associated Igs, and the amounts released per platelet were independent of the platelet concentration over a wide range of concentrations. This procedure is suitable for sensitive, reproducible, and specific quantitation of large numbers of samples. Washed platelets from 13 normal donors contained the following components (expressed in femtograms per platelet, mean +/- 2 SEM): IgG, 1.40 +/- 0.26; IgA, 0.72 +/- 0.36; IgM 0.078 +/- 0.036; albumin 7.7 +/- 1.5. Immunoglobulins and albumin eluted from the platelets of ten ITP patients (two in remission), expressed as femtograms per platelet, mean (range), were: IgG 104 (0.3 to 750); IgA 90 (0.9 to 715); IgM 162 (1.2 to 1,300); and albumin 34 (6.8 to 199). All platelet-associated Igs from thrombocytopenic ITP patients were found to be elevated twofold to 2,300-fold with one Ig class occasionally elevated 50-fold to 100-fold higher than the others. A similar group of ten thrombocytopenic ITP patients was found to have twofold to 26-fold elevations of platelet- associated albumin. This demonstration of increases in multiple classes of Igs as well as serum albumin associated with platelets from ITP patients suggests that some nonimmune process may be contributing to the phenomenon of increased platelet-associated proteins in ITP.

scholarly journals Studies on Thrombopoiesis

Blood ◽  
1957 ◽  
Vol 12 (6) ◽  
pp. 520-528 ◽  
Author(s):  
G. IZAK ◽  
D. NELKEN ◽  
MISS A. HERZOG
Keyword(s):  
Idiopathic Thrombocytopenic Purpura ◽  
Culture Media ◽  
Tissue Cultures ◽  
Continuous Observation ◽  
Healthy Individuals ◽  
Chronic Idiopathic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura ◽  
Platelet Production ◽  
Bone Marrow Cultures ◽  
Marrow Cultures

Abstract Thrombocytopoiesis was studied by direct, continuous observation in tissue cultures of bone marrows taken from three patients with chronic idiopathic thrombocytopenic purpura and from normals following the addition of a potent anti-platelet serum to the culture media. The process of platelet production was similar in these two conditions and followed the pattern observed in bone marrow cultures of healthy individuals. The breakdown of megakaryocytes of patients with idiopathic thrombocytopenic purpura and those treated with anti-platelet serum was greatly accelerated. No morphologic evidence of injury to the megakaryocytes was present. The platelets produced showed degenerative changes, they were agglutinated and underwent phagocytosis by the myeloid elements and reticulum cells were present in the cultures.

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