Case Report. Idiopathic Thrombocytopenic Purpura: Overview And Report Of Two Cases.

Transcription

1 JPMCP Medicine Case Report Dr. Abhay Kulkarni, Dr. Sagar Khairvar, Dr. Rajendra Birangane, Dr. Sanjeev Onkar Kulkarni A, Khairvar S, Birangane R, Onkar S. Idiopathic thrombocytopenic purpura: Overview and report of two cases. J Periodontal Med Clin Pract 2015; 02:36-41 Affiliation 1. Assistant Professor, Department Of Oral Medicine and Radiology, PDU Dental College, Solapur, 2. PG Student, Department Of Oral Medicine and Radiology, PDU Dental College, Solapur, 3. Prof. & HOD, Department Of Oral Medicine and Radiology, PDU Dental College, Solapur, 4. Reader, Department Of Oral Medicine and Radiology, PDU Dental College, Solapur, Maharashtra, India. Corresponding Author: Dr. Abhay Kulkarni Assistant Professor, Department Of Oral Medicine and Radiology, PDU Dental College, Solapur, Abstract: Idiopathic thrombocytopenic purpura (ITP) also called as Immune thrombocytopenic purpura is an acquired autoimmune disorder in which auto-antibodies are made against platelets leading to increased platelet destruction. Immune thrombocytopenia, increased splenic sequestration, infections such as dengue fever, decreased bone marrow production and adverse drug reactions are the major causes of ITP. Patients with ITP often present with clinical manifestations of bruising and bleeding. Albeit these manifestations are detected most frequently on the skin; on the lower extremities, the oral cavity also may exhibit signs of petechiae, purpura and bleeding. Haemorrhagic bullae of mucous membranes in the oral cavity can indicate the existence of severe thrombocytopenia. We report two female cases presented with oral features of petechial haemorrhage, pigmented brownish black macules with bleeding, indicative of a bleeding disorders. Laboratory test results revealed the idiopathic thrombocytopenia which necessitates the importance of diagnosis of oral manifestations of thrombocytopenia. KEYWORDS: Idiopathic thrombocytopenic purpura, Immune thrombocytopenic purpura, petechiae, thrombocytopenia, Bleeding disorders. Introduction Platelets (thrombocytes) derived from megakaryocytes have an elemental function in the blood clotting system and contribute in the initial 36

2 JPMCP stage of the haemostatic process through adhering to the collagen and aggregating to form the clot thus, disorders accompanied by decreased platelet number or platelet dysfunction may give rise to [2] pathologic bleeding states. Platelet count below [3] 50,000 cu mm is referred as thrombocytopenia. Normal range is between 1,50000 to 4,50000 cu mm. [2, 3] Thrombocytopenia or decrease platelet function can take place in the course of a variety of mechanisms, including autoimmune destruction, infections such as dengue fever, spleen sequestration, tumor cell infiltration of bone [4] marrow, and many adverse drug reactions. Idiopathic thrombocytopenic purpura (ITP) also known as immune thrombocytopenic purpura is an acquired autoimmune disease in which there is accelerated platelet destruction due to [5] autoantibodies (7S IgG) made against platelets. As the disorder is of immunological nature, in which the host's platelets are destroyed by host's immune system so the many authors recommend t h e t e r m i d i o p a t h i c b e r e p l a c e d b y [6] autoimmune. Medicine It occurs in two clinical types; acute self-limiting form which affects exclusively children with the history of severe viral infection and chronic form seen mostly in adults. Acute ITP is common in both sexes and has a peak incidence [3, 4, 5] of 3-5 years. Chronic form is commonly seen in [7] years adults with female predilection (3:1). Evidences available suggestspontaneous remission [8] is seen in only 5% of adults with chronic ITP. Spontaneous Gingival bleeding or due to minor traumatic event like tooth brushing, hard food biting and flossing, is mostly the first sign of [4] thrombocytopenia. [1] The soft palate and buccal mucosa, may display ecchymoses and petechial haemorrhages. In cases with very low platelet counts deep red to black haemorrhagic bullae may [9] occur. This paper presents two rare cases of idiopathic thrombocytopenic purpura in females with different intraoral presentations. CASE REPORT: Case 1: A 40 year old female reported to the Dept. of oral medicine and radiology with a complaint of brownish red pigmentations in her mouth and all over the body. She noticed haemorrhagic petechiae and red brown spots in mouth 2 weeks back. She also noticed reddish pigmented spots on back, arms and both legs and bleeding through mouth while gargling and brushing. Physical examination revealed a well-nourished, well-developed, cooperative woman in no acute distress. Her vital signs were stable. On extra oral examination multiple small reddish brown haemorrhagic macules of approximately 1-2 cm in diameter seen on both arms, both legs and slightly larger in size on back, [Figure 1, 2, 3]. Bluish discolouration of sclera also noted, [Figure 4]. Intraoral examination revealed single reddish haemorrhagic papule of approximately 1x1.5 cm in size noticed on lower labial mucosa, [Figure 5] and also same presentation which is of smaller in size noted on left buccal mucosa [Figure 6], soft palate, left lateral border of tongue, [Figure 7] and gingiva, [Figure 8]. Small tiny multiple haemorrhagic spots also seen on ventral surface of tongue and floor of mouth, [Figure 9] In the differential diagnosis acute leukemia and idiopathic thrombocytopenic purpura (ITP) were considered in light of the petechial and ecchymotic lesions, suggesting a hemorrhagic diathesis. 37

3 JPMCP Medicine Laboratory results revealed that haemoglobin, haematocrit, and white blood cell counts, as well as prothrombin and partial thromboplastin times, were all within reference range. The platelet count, however, was drastically low at cu mm (reference range, 1, , cu mm). Peripheral smear showed normal size platelets. Hence on the basis of all investigatory findings Figure 1: Reddish brown haemorrhagic macule on arms. Figure 3: Multiple, reddish brown macules seen on back. Figure 5: Single, reddish haemorrhagic papule of 1x2 cm in size on lower labial mucosa. 38 patient was diagnosed with ITP and sent to hematologist for further examination. In treatment she received 1 U of platelets, intravenous immunoglobulin G, and prednisone starting at 80 mg/d. Patient was followed up after 2 weeks her platelet count was raised to upto 3 135,000 mm Figure 2: Red haemorrhagic spots on both the legs. Figure 4: Cyanosis (Bluish discolouration of sclera). Figure 6: Single, round, red papule on left buccal mucosa.

4 JPMCP Medicine Figure 7: Reddish haemorragic papule on left lateral border of tongue; easy to bleed. Figure 8: Generalised gingival with bleeding. Inflamed Figure 9: Small tiny multiple haemorrhagic spots on ventral surface of tongue and floor of mouth. Case 2: Another 65 year old female reported to the Dept. of oral medicine and radiology with a complaint of bleeding from mouth and brownish pigmentations on all over the body. She noticed bleeding from oral mucosa and tongue and also noticed haemorrhagic patches on tongue and floor of the mouth. She also noticed brownish pigmented patches of varying sizes on face, arm and neck. On extra-oral examination single large brownish black pigmented macule of approximately 2x3 cm in size and also few small sized brownish black macules noted on middle third region of right side of face, [Figure 10]. These types of large sized macules are also noted on clavicular region on neck and forearms, [Figure 11, 12]. On intraoral examination large reddish haemorrhagic papule of approximately 2x2.5 cm in size noticed on right lateral border of tongue and also small sized papules noticed on right and left lateral border of tongue and floor of mouth, [Figure 13]. On 39 palpation these papules were easy to bleed. Differential diagnosis includes metastatic melanoma because of the shiny black appearance of the papules. Acute leukaemia, Actinomycosis, histoplasmosis, aspergillosis, and cryptococcosis were included in the differential diagnosis because oral granulomatous papules also occur in these diseases. In laboratory results the platelet count was significantly low at cu mm (reference range, cu mm). Normal size platelets showed in peripheral smear. Hence final diagnosis of Idiopathic thrombocytopenic purpura was concluded and referred to haematologist. In treatment Corticosteroids is been prescribed; Prednisone 80mg/day. 1 U of platelets was given and also intravenous immunoglobulin G. Patient was followed up for 1 year within 2 weeks her 3 platelet count was raised to 1, mm and subsequently oral mucosal lesions are also been subsided.

5 JPMCP Medicine Figure 10: Single large brownish black pigmented macule of approximately 2x3 cm in size with few small sized brownish black macules on right side of face. Figure 12: Brownish black large macule on forearm of approximately 2x4 cm in size. Discussion: ITP is diagnosed by the exclusion of other diseases associated with low platelet count. Diagnosis is best achieved by proper history, physical examination, complete blood count and peripheral [7] smear examination. About 30% to 40% of adults having I T P have no symptoms. Clinical manifestation of ITP shows great disparity. Frequent muco-cutaneous lesions seen in ITP are Petechiae, purpura, easy bruising and hematoma. Rare manifestations are hematuria, GI bleeding and intracranial haemorrhage although intracranial [3, 5] haemorrhage is most common cause of death. Petechiae and ecchymoses are tends to develop on 40 Figure 11: Large sized brownish black macules on clavicular region on neck. Figure 13: large reddish haemorrhagic papules of approximately 2x2.5 cm in size on right lateral border of tongue and floor of mouth. skin. Purpura likely to be form primarily in areas of increased venous pressure, such as extremities like legs. Unprompted gingival bleeding occurs on [7,9] decreased platelet count less than 20,000 cu mm. ITP can exist in a subclinical form, and severe haemorrhage due to lacerations or minor medical and dental surgical procedures can be its first [1] clinical manifestation,. Laboratory results are generally suitable indicators of ITP. Haemoglobin count is typically normal unless significant haemorrhage associated with thrombocytopenia has resulted in anaemia. The WBC count usually is [5] in normal reference range,. Other findings are prolonged bleeding time, poor clot retraction;

6 JPMCP Medicine normal clotting time and positive tourniquet test are other findings. Peripheral smear shows reduced platelet count and is crucial to exclude thrombotic [7] thrombocytopenic purpura and acute leukemia. Early treatment of choice for the patient having very low platelet counts value like 10,000 to 20,000 cu mm leading to intense bleeding is with Intravenous Immunoglobulin (IV-Ig) alone or in c o m b i n a t i o n w i t h I n t r a v e n o u s m e t h y l prednisolone. In conditions of non-emergency initial treatment can be prednisolone 1-2 mg/kg/day). For patients intolerant to corticosteroids, intravenous IV Ig anti-d can be [3, 9] given. The newer therapeutic modalities with varied mechanisms of action, for example rituximab, anti-d, and thrombopoietin like agents [9] can be administered in treatment of ITP. References: 1. Guilherme Martins, Fabrício Gomes Rozas, Marina de Oliveira Ribas, et al. Idiopathic thrombocytopenic purpura and oral surgery: case report. Rev Clín Pesq Odontol jan/apr; 6: Amit Byatnal, Neha Mahajan, Shrinivas Koppal et al. Unusual yet isolated oral m a n i f e s t a t i o n s o f p e r s i s t e n t thrombocytopenia A rare case report. Braz J Oral Sci. 12(3): Dr. Prashant Salve, Dr. Nandita Gupta. Oral Manifestations of Idiopathic Thrombocytopenic Purpura: A Rare Case Report. International journal of scientific research. Volume: 3, Issue: 8, Aug LT Amy E. Helms, Robert I. Schaffer. Idiopathic Thrombocytopenic Purpura with Black Oral Mucosal Lesions. Cutis. 2007; 79: S i m o n K a r p a t k i n. A u t o i m m u n e (idiopathic) thrombocytopenic purpura. Lancet 1997; 349: Sunitha M, R Rajesh. Idiopathic Trombocytopenic Purpura: Overview with report of a case. Journal of Indian Academy of Oral Medicine and Radiology. Julyseptember 2011, 23(3): George JN, Wolf SH, Raskob GE, et al. Idiopathic Thrombocytopenic Purpura: A practice guideline developed by explicit methods for the American Society of Hematology. Blood 1996:88: Schlosser BJ, Pirigyi M, Mirowski GW. Oral manifestations of hematologic and nutritional diseases. Otolaryngol Clin N Am. 2011; 44: B u s s e l J. Tr e a t m e n t o f i m m u n e thrombocytopenic purpura in adults. Semin Hematol 2006; 43:S3 10. Competing interest / Con ict of interest The author(s) have no competing interests for nancial support, publication of this research, patents and royalties through this collaborative research. All authors were equally involved in discussed research work. There is no nancial con ict with the subject matter discussed in the manuscript. Source of support: NIL 41 Copyright 2014 JPMCP. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.