Hazel Joyce D.

Moleta

LITERATURE NO. 1) The Frequency and Clinical Significance of Thrombocytopenia Complicating Critical Illness

FINDINGS -Thrombocytopenia appears to increase the risk of death -The main concern for the critically ill thrombocytopenic patient is bleeding -Thrombocytopenia was an independent predictor of death among critically ill patients in most studies

CLAIMS -Literature nos. 2 & 4 presents similarity that ITP are most common in children. - Literature nos.2 & 7 presented that VWFs function affect in Platelet and hemostasis - Literature nos. 2 & 3 proved that Autoimmune diseases and bleeding disorders have genetic linkage

EVIDENCE -6 out of 8 studies using multivariate analysis found that thrombocytopenia increased the risk of death -5 studies of thrombocytopenia in the ICU reported bleeding outcomes -5 out of 8 studies were more likely to receive RBC transfusions -6 out of 8 studies found that thrombocytopenia was an independent predictor of death -Idiopathic thrombocytopenic purpura is usually a temporary disorder, with 80 to 90 percent of children recovering within 6-12 months, usually within a few weeks

2) Evaluating the child with purpura

3) Autoimmune diseases in a Nigerian woman-A case report

-both genders are equally affected -anemia with thrombocytopenia indicates leukemia -platelet disorder present vasculitic disorder including VWF -chronic idiopathic thrombocytopenic purpura is more likely in teenage girls and children -genetic counseling is useful in families with inherited bleeding disorder -genetic influence via the histocompatibility leukocyte antigen is prominent among the autoimmune disease

- drug of choice common in ITP is steroids based on literature nos. 6, & 4 however it was contrast in literature no.9 because the study shown that there is a remission in taking prednisone -ITP is common in South Africa based on literature nos.10 & 4 - Weakness, bruising and bleeding is common symptoms of ITP that was cited on literature nos. 4 & 8

-presence of combinations of autoimmune disease spectrum in this case as well as the presence of vitiligo in her daughter may

Hazel Joyce D. Moleta

-Thrombocytopenia/ ITP is a rare condition and its serious if not treated correctly

4) A survey of the management of idiopathic thrombocytopenic purpura in South Africa: Do we need guidelines for developing countries 5) The Diagnostic dilemma of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in the obstetric triage and emergency department: lessons from 4 tertiary hospitals 6) International consensus report on the investigation and management of primary immune thrombocytopenia

-steroids were the first line of choice in treating ITP -ITP is the most common bleeding disorder of childhood -ITP is the most common bleeding disorder in children in south africa -TTP /HUS are both rare -the most common reported symptoms include weakness, bleeding, and easily bruisabilty

well suggest a genetic link -the roles of genetic, environmental and immunoregulatory factors were outlined by Van Noort et al, when they reinforced the multifactorial origin of autoimmunity -vast majority of doctors would prescribe steroids or immunoglobulins -most cases are treated in academic centers indicating a high rate referrals -they afflict 1 in 100,000 pregnancies to 1:1,000,000 people in general population

-ITP is an acquired immune mediated disorder characterized by isolated thrombocytopenia, defined as a peripheral blood platelet, count less than 100X10g/L -incidence in adults is approximately equal for the sexes -Corticosteroids are the standard initial treatment

-a positive direct antiglobulin test (DAT) was found in 22% of 205 patients with ITP

Hazel Joyce D. Moleta

7) The Laboratory diagnosis of Platelet disorder

8) Current Concepts in Thrombotic Thrombocytopenic Purpura

9)Autoimmune thrombocytopenic Purpura and Common Variable Immuodeficiency: Analysis of 21 Cases and review of the literature 10) Platelet Counts and mean Platelet Volume Amongst elderly Nigerians

-Prednisone is the standard initial first line therapy for ITP patients - an abnormality of VWF has bleeding symptoms very similar to platelet dysfunction, and evaluation for von Willebrand disease should be included in the initial evaluation of a possible platelet disorder -Patients typically present with weakness, pallor,petechiae, headache or subtle mental changes.If not treated, the disease may rapidly deteriorate to stupor, coma or cardiac arrest -steroids seem to have the same efficacy as in idiopathic AITP, but the increased risk of severe infections must be taken into considerations -AITP I not prevented by IVIG substitutive therapy -healthy elderly and young adult control females having higher platelet counts than the elderly and young adult control males -there is need for separate values for platelet count and mean platelet volume for the elderly

-platelets adhere to VWF and to fibrinogen through glycoprotein receptor according to Arch Pathol Lab Med-Vol 126, February 2002 Laboratory Diagnosis of Platelet Disorders-KottkeMarchant et al -TTP ha reduced its case fatality from 90% to 10%20% by the use of plasma infusion and plasma exchange

-62% were in treatment free remission and 23% where in remission while on prednisone

-Lazomo et al(1998) reported sex difference in platelet count with higher values in females than in males -study suggests that there is significant difference in platelet count between the healthy elderly subjects and

Hazel Joyce D. Moleta

young adult control