Idiopathic Thrombocytopenic Purpura (ITP) is a hemorrhagic condition, characterized by the abnormally low levels of platelets in the blood. Although ITP is known as an idiopathic condition, it is widely believed that it is an autoimmune disorder, in which autoantibodies are produced against the platelets. Two major forms of ITP are recognized. The acute form is usually seen in children, and lasts for less than 6-months, while the chronic form, usually seen in adults is more severe, and may last for years. Characteristic symptoms include purpura (bruises), petechia (reddish-purplish spots on the skin resembling rashes), increased menstrual flow in women, bleeding from gums and nose, and blood in urine and stools. Serious complications of the condition include profuse bleeding from cuts and the risk of intracranial hemorrhage. A lot of people with this condition do not show any physical outward symptoms, and are discovered with mild thrombocytopenia, only when their blood profile is studied for some reason.
The acute form of ITP is not very serious and most children do not necessarily require any form of treatment. However, a short treatment with corticosteroids or gamma globulin may help. For chronic cases of ITP, again corticosteroids, like prednisolone, are used. Splenectomy (surgical removal of the spleen) is another option, whereby the immune system can be regulated. It is important to note, however, that these treatment options are not without their associated side effects. Long-term usage of corticosteroids carry the risk of developing osteoporosis, cataracts, and muscle wasting, while splenectomy increases the risk of infections.
The familial nature of ITP is demonstrated by the study of a number of familial cases of the condition, although their occurance is much fewer compared to sporadic cases. While the exact genetic cause is unkown, a recent study has shown the presence of an ORF allele of the FCGR2C gene among ITP patients in a proportion higher than the average.
Afifi et al. (1981) studied childhood idiopathic thrombocytopenic purpura in 160 patients from Egypt, Saudi Arabia, Qatar and North Sudan. The disease revealed a heterogeneous spectrum that included three distinct clinical forms: (a) the acute self-limited form, (b) the intermediate form, and (c) the chronic adulthood-like form. The relative proportions of these forms were 40, 15, and 45%, respectively. The chronic form shows limited response to steroids, and runs a platelet count less than 100,000 microliters for more than 1 year, with a tendency for later spontaneous elevation in platelet counts during the first few years of a long follow-up. The intermediate form shows a transient steroid-induced complete remission giving place to widely fluctuating platelet counts above and below 100,000 microliters once the steroid dosage is reduced to maintenance levels. Platelet counts in excess of 100,000 microliters were achieved in this group by extending steroid maintenance therapy for 6-9 months. In spite of a tendency to chronicity and partial resistance to steroids in the intermediate and chronic forms, the overall response to steroids was enough both to reduce the number of cases requiring splenectomy to 15%, and to prevent the development of major complications in all the children.
Hijazi et al. (1995) studied 56 Arab children (27 males, 29 females) aged 2 to 12 years with idiopathic thrombocytopenic purpura (ITP). Out of the 56 children, 31 were found to suffer from splenomegaly and of those, iron deficiency anemia was found in 84% in contrast to 48% in those without splenomegaly. The prevalence was calculated in patients with and without splenomegaly without finding any significant distinction in the prevalence among beta-thalassemia trait or history of previous viral infection. Hijazi et al. (1995) revealed a higher prevalence of splenomegaly among Arab children with ITP with a possibility of a link to iron deficiency and proposed carrying out bone marrow examination for exclusion purposes of more serious illnesses such as leukemia.
Abou Shanab (1996) followed up 20 children (12 males and 8 females) with acute immune (idiopathic) thrombocytopenic purpura (ITP) from May 1985 to November 1994. Most of the cases suffered a degree of mucocutaneous bleeding without hepatosplenomegaly and received conservative treatment. Spontaneous remission occurred in all cases. Two years later, Abou Shanab (1998) presented the cases of 17 patients; six males and 11 females with chronic immune thrombocytopenic purpura. These were followed up for periods ranging between 7-months and 7-years, during which no deaths nor intracranial hemorrhages were recorded. Initial platelet counts were < 10 X10^9/L in eight cases and 10-30 X10^9/L in the other nine. In the study period, five cases experienced spontaneous remissions. [Abou Shanab OA. Childhood acute immune (idiopathic) thrombocytopenic purpura in Kuwait: the experience of Al-Sabah hospital. Kuwait Med J. 1996; 28(4):451-4.] [Abou Shanab OA. Childhood chronic immune thrombocytopenic purpura; how far should we treat? Kuwait Med J. 1998; 30(3):196-200.]
Hegazi et al. (2008) reported on the co-existence of common variable immunodeficiency (CVID) with idiopathic thrombocytopenic purpura (ITP). No further details could be obtained on this case.
Wali et al. (2002) prospectively studied the effect of high dose dexamethasone given in pulses for children with refractory chronic idiopathic thrombocytopenic purpura (ITP). All children (13 children - five boys and eight girls) diagnosed with chronic ITP according to established clinical criteria and bone marrow aspirate (which showed normal or increased megakaryocytes) during a period of 18 months were included in the study. These children did not have a sustained response to other treatment modalities used singly or in combination. There was no positive family history of thrombocytopenia. The children were started on oral Dexamethasone 40mg/m2 in single morning dose for four days every four weeks for six months, along with antacids and/or H2 blockers before each dose. All the identified patients completed the six cycles of therapy and tolerated the side effects which were not severe enough to discontinue the therapy. An excellent immediate response to therapy was noticed with mean platelet count of 158.92x10(power 9)/L on the fourth day of the cycle. Three patients were complete responders by the end of the six cycles and they continued to be so till the end of two years, while out of four partial responders at the end of the therapy, only three continued to be so for the whole two years. Seven patients were classified as failures by the end of two years post therapy.
Al-Mulla et al. (2009) studied the pattern of idiopathic thrombocytopenic purpura (ITP, acute/chronic) and the presenting features and clinical characteristics of the disease in 50 children below 14 years of age (31 Qatari and 19 non-Qatari) through a retrospective, descriptive study conducted from 2000 to 2005 in the Pediatric Department of the Hamad General Hospital (HGH), Hamad Medical Corporation. Among the studied children, 62% were diagnosed with acute ITP and 38% with chronic ITP. Acute ITP was more prevalent in males (64.5%) when compared with females (35.5%), whereas for chronic ITP, nearly an equal distribution was found in males (57.9%) and females (42.1%). In the comparison of age groups, the highest number of children with ITP was found in the 1-4-year group (60%), followed by children in the 5-9-year group (22%). No significant seasonal occurrence and familial predisposition were observed in this study. Preceding viral infection was common in both acute (71%) and chronic (63.2%) ITP cases; 68% of were treated with intravenous immunoglobulin (74%). Al-Mulla et al. (2009) concluded that children with ITP showed a platelet count below 20x10(9)/L at the time of presentation. Most of the studied children revealed a high incidence of ITP among Qatari children and highlights the situation of ITP and may help establish national guidelines for the investigation and management of childhood ITP in the country.
[See also: Egypt > Afifi et al., 1981].
[See: Egypt > Afifi et al., 1981].