CASE REPORT ACUTE IDIOPATHIC THROMBOCYTOPENIC PURPURA

Presented by: Pasca Sari Nauli Tamba R.D. Tiopan Napitupulu Supervisor : dr. Rita Evalina, Sp. A (K)

FAKULTAS KEDOKTERAN UNIVERSITAS SUMATERA UTARA MEDAN 2012

CASE REPORT ACUTE IDIOPATHIC THROMBOCYTOPENIC PURPURA (ACUTE ITP) Division of Pediatric Allergy and Immunology Presentator: Pasca Sari Nauli Tamba R.D. Tiopan Napitupulu Supervisor : dr. Rita Evalina , Sp.A (K) Introduction ITP, which variably been defined as idiopathic thrombocytopenic purpura, and, most recently, immune thrombocytopenia is an autoimmune disorder characterized by immunologic destruction of otherwise normal platelets most commonly occurring in response to an unknown stimulus. ITP may occur in isolation (primary) or in association with other disorders (secondary). Secondary causes include autoimmune diseases (particularly the antiphospholipid antibody syndrome), viral infections (including hepatitis C [HCV] and human immunodeficiency virus [HIV]), and certain drugs.1 ITP is usually an acquired disorder in which platelets are coated (opsonized) with antiplatelet autoantibodies and removed prematurely by the reticuloendothelial system, predominantly the spleen, leading to a reduced peripheral blood platelet count.2 Childhood ITP is generally termed acute since the illness is seasonal, typically follows a trivial viral infection or vaccination, is transient in most cases and requires no treatment, with spontaneous recovery in 80% of cases.1 One to 4 wk after exposure to a common viral infection, a small number of children develop an autoantibody directed against the platelet surface. A preceding history of a viral illness is described in 5065% of cases of childhood ITP. The reason why some children respond to a common infection with an autoimmune disease remains unknown.3 The estimated incidence in children is approximately 1.9 to 6.4 cases per 100,000 per year and for adults 3.3 per 100,000 per year.4 Retrospective data, consensus statements, expert opinion and textbooks suggest that childhood and adult onset ITP have distinctly different laboratory findings and clinical features.4 The classic presentation of ITP is that of a previously healthy 14 yr old child who has the sudden onset of generalized petechiae and purpura. The parents often state that the child was

fine yesterday and now is covered with bruises and purple dots. Often there is bleeding from the gums and mucous membrane, particularly with profound thrombocytopenia (platelet count < 10 109/L). There is a history of a preceding viral infection 1 to 4 wk before onset of the thrombocytopenia. The physical examination is normal other than the finding of petechiae and purpura. When the onset is insidious, especially in an adolescent, the possibility of chronic ITP or that thrombocytopenia is a manifestation of a systemic illness such as systemic lupus erythematosus (SLE) is more likely.3 The diagnosis of ITP remains clinical, and one of exclusion. Standard investigation includes a full blood count (isolated thrombocytopenia), blood film (to ensure no red cell fragments, leukemia, parasitic infections) and autoimmune profile (to exclude secondary cause). A bone marrow examination is often carried out in adults but not usually in children, and will usually show normal or increased megakaryocytes in an otherwise normal marrow. Immunological assays have been devised, including platelet-associated IgG or IgM and monoclonal antibody immobilization of platelet antigens, but these do not alter the management and are of debatable value.2 Initial treatment options for ITP include Intravenous immunoglobulin (IVIG), which in a dose of 0.81g/kg/day 12 days, induces a rapid rise in platelet count (usually >20 10 9/L) in 95% of patients within 48hr, Prednisone, in doses of 14mg/kg/24hr of prednisone appear to induce a more rapid rise in platelet counts than in untreated patients with ITP, and if reasonable, an IV Anti- D Therapy, but its role in initial therapy of acute ITP is under investigation. Each of these medications may be used to treat exacerbations of ITP, which commonly occur several weeks after an initial course of therapy.3 The prognosis of acute ITP in children is good. Approximately 83% of children have a spontaneous remission, and 89% of children eventually recover. More than 50% of patients recover within 4-8 weeks. Only approximately 2% of patients die.5

CASE Objective The aim of this paper is to report a case of Acute Idiopathic Thrombocytopenic Purpura in an 8 years 2 months old girl. Case R, an 8 years and 2 month old girl was admitted to Haji Adam Malik Hospital on July 31st, 2012 with a chief complaint of history of bloody vomiting 10 days ago. Vomiting was only once, blackish color, with clots and about 120 ml in volume. History of previous bloody vomiting was found a month ago, only once, and due to her illness, the patient was hospitalized for 3 days at RSIA Subullusalam. Epistaxis was also found 10 days ago. History of previous epistaxis was found a month ago. The epistaxis resolved after being hospitalized at RSIA Subullusalam but it reoccurred 2 weeks later. Epistaxis was exacerbated by the presence of fever. History of bleeding gums was not found. Bruises were found since 1 month ago on the lower extremities. Bruises are currently fading now. History of bruising after the patient bumps into objects was found. History of recurrent fever was found for 2 months, subfebris, and was relieved by medication. Cough and cold was not found. Micturition and defecation (+) normal. Weight loss was not found. History of living in an industrial area was not found. The patient is living near an area of fertilizer storage, about 10 m from her house, since 4 years ago. The patient uses water from a well in her household. A family history of similar illness was not found. History of blood transfusion was found in June when patient was in another hospital. History of previous illness : She was referred DHF Grd II-III History of previous medication : Blood transfusion on June 2012 in RSUD Rimosingkil, RL infusion, cefotaxim, ranitidine, metilprednisolone injection, antacid Physical Examination Presence Status : Sensorium : Compos Mentis, Temperature: 37C Anemic (+), Dyspnea (-), Cyanotic (-), Edema (-), Icteric (-). from RSIA Subullusalam - RSUD Rimosingkil with differential diagnosis: Suspect ITP,

Weight: 18 kg Length: 122 cm BW/BL: 95.6% Head : Eye : Light reflexes (+/+), isochoric pupil, pale conjunctiva palpebra inferior (+/+) Ear and Mouth: within normal limit; Nose: nasal flare (+) Neck Thorax : Lymph node enlargement (-) : Symmetrical fusiform , retraction (-) HR: 95 bpm, regular, murmur (-) RR : 25 bpm, regular, ronchi (-/-), Abdomen Genitalia Extremities : Soepel, peristaltic (+) N, Hepar/Lien: non palpable : Female, within normal limit : Pulse : 95 bpm, regular, adequate pressure/volume, CRT<3, warm, edema (-), haematom on the lower extremities (+/+)

Laboratory Result (From local hospital [RSUD Rimosingkil]): Date 23 July 2012 Test Hemoglobin (Hb): 11,1 Hematocrite (Ht) : 32,9 Leukocyte : 9,1 Platelet : 40 Hemoglobin (Hb): 11,3 Hematocrite (Ht) : 32,9 Leukocyte : 9,7 Platelet : 34 IgG/IgM: -/Unit g% % 3 10 /mm3 103/mm3 g% % 103/mm3 103/mm3

25 July 2012

30 July 2012

(RSUP Haji Adam Malik , August 1st 2012) : Test Complete Blood Count Hemoglobin (Hb) Results 11.50 Normal Value 11.3 14.1 g %

Erytrocyte (RBC) Leukocyte (WBC) Hematocrite Trombocyte (PLT) MCV MCH MCHC RDW MPV PCT PDW Neutrofil Limfosit Monosit Eosinophil Basophil Differential Diagnosis -

4.04 10.42 34.00 51 84.20 28.50 33.80 13.00 12.60 0.06 17.7 44.30 39.00 10.20 5.40 1.100

4.40 4.48 106/mm3 4.5 13,5 103/mm3 37 41 % 150 450 103/mm3 81 95 fl 25 29 pg 29 31 g% 11.6 14.8 % 7.0 10.2 fl % fl 37 80 % 20 40 % 28% 16% 01%

Idiopathic Thrombocytopenic Purpura Autoimmune Thrombocytopenic Purpura Dissaminated Intravascular Coagulation Acute Leukaemia

Working Diagnosis Idiopathic Thrombocytopenic Purpura Treatment -

Activity: Bedrest Diet: MB 1400 kcal with 40 gr protein IVFD D5% NaCl 0,45% 20 microdrips/minute

Planning
-

Observe for any signs of bleeding Serial peripheral blood smear or serial complete blood count if reasonable

Follow Up Date Complaints Physical Findings Fever Pale Bleeding Lymph node others Laboratorium Hb Ht Leukocyte Platelet LED Eosinophil Basophil Neutrophil Lymphocyte Monocyte Reticulocyte Peripheral Blood Smear Therapy 1/8/2012 Bruises(+) Sens:CM T: 36,5C HR: 97x/i RR: 26 x/i + 11,5 34 10,42 51 5,4 1,1 44,3 39 10,2 1,69 2/8/2012 Bruises(+) Sens: CM T: 37 C HR: 96 x/i RR: 28 x/i + 3/8/2012 Bruises(+) Sens: CM T: 36,8C HR: 98 x/i RR: 26 x/i 4/8/2012 Bruises (+) Sens: CM T: 37C HR: 96 x/i RR: 28 x/i 5/8/2012 Bruises(+) Sens: CM T: 36,5C HR: 95 x/i RR: 24 x/i 6/8/2012 Bruises(+) Sens: CM T: 37C HR: 96 x/i RR: 26 x/i 12,10 35,40 10,12 15 7,0 0,6 57,7 27,3 7,4

IVFD D5% NaCl 0,45% 20 mcd/min Observe for any signs of bleeding 7/8/2012 Bruises (-)

IVFD D5% NaCl 0,45% 20 mcd/min Observe for any signs of bleeding

IVFD D5% NaCl 0,45% 20 mcd/min Observe for any signs of bleeding 8/8/2012

IVFD D5% NaCl 0,45% 20 mcd/min

IVFD D5% NaCl 0,45% 20 mcd/min

IVFD D5% NaCl 0,45% 20 mcd/min

Note

Observe for any signs of bleeding 9/8/2012 Bruises

Observe for any signs of bleeding

Observe for any signs of bleeding

Date Complaints

10/8/ 2012 Bruises

11/8/ 2012 Bruises

12/8/ 2012 Bruises

Bruises (-)

Physical Findings

Sens:CM T: 37C HR: 96x/i RR: 32 x/i

Sens: CM T: 36,7 C HR: 90 x/i RR: 24 x/i

(-) Sens: CM T: 36,7C HR: 94 x/i RR: 24 x/i -

(-) Sens: CM T: 37C HR: 96 x/i RR: 26 x/i -

Fever Pale Bleeding Lymph node others Laboratorium Hb Ht Leukocyte Platelet LED Eosinophil Basophil Neutrophil Lymphocyte Monocyte Reticulocyte Peripheral Blood Smear

11,5 34 10,42 51 5,4 1,1 44,3 39 10,2 1,69

(-) Sens: CM T: 36,2C HR: 99 x/i RR: 29 x/i -

(-) Sens: CM T: 36,3C HR: 96 x/i RR: 28 x/i 12,10 35,40 10,12 15 7,0 0,6 57,7 27,3 7,4

Platelet: 16.000

Platelet: 23.000

Therapy

Inj. Methylpredniso lone (HD:30mg/kgB W/d/IV for 2 days) 540 mg/IV/d 1st day Observe for any signs of bleeding

Inj. Methylpredniso lone 540 mg/IV/d 2nd day

Prednison e2 mg/kgB W/d for 1 weeks 36 mg 3-2-2 1st day Observe for any signs of bleeding

Predniso ne 3-2-2 2nd day

Predniso ne 3-2-2 3rd day

Predniso ne 3-2-2 4th day

Note

Observe for any signs of bleeding

Check CBC/ week

Check CBC/ week

Check CBC/ week

Date Complaints Physical Findings

13/8/2012 Bruises(-), Fever (-), Pale (-) Sens:CM T: 37C HR: 98x/i RR: 26 x/i

14/8/2012 Bruises(-), Fever (-), Pale (-) Sens: CM T: 36,8 C HR: 98 x/i RR: 28 x/i 14,2 40,20 25,39 211 0,3 0,1 73,2 18,2 8,2

Laboratorium Hb Ht Leukocyte Platelet LED Eosinophil Basophil Neutrophil Lymphocyte Monocyte Reticulocyte Therapy Note

Prednisone 3-2-2 5th day Check CBC/week

Prednisone 3-2-2 6th day R/ Outpatient(PBJ)

Discusion .next!!!!!!