The one where all paths point to ITP

Date

2023

Authors

Cruz, Krystal
Hasan, Aya

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Abstract

Background:

Immune Thrombocytopenic Purpura (ITP) is an autoimmune condition where antibodies against platelets can eventually lead to sequestration by the spleen and liver inducing a thrombocytopenia. Although primary idiopathic ITP is most common, secondary ITP associated with a trigger such as malignancy, infection, and drugs have also been reported.

Case Description:

We report a case of a 66 year old male status post two years of chemoradiation for invasive squamous cell carcinoma of oropharynx. This is a unique case where our patient presented to the ED with vomiting, generalized weakness, and a low grade fever. Initial physical exam was negative for mucosal bleeding and petechial rash. Initial labs indicated severe thrombocytopenia with platelets as low as 36,000/UL, AKI with creatinine of 3.73, and elevated LFTs. There was concern for TTP vs. ST-HUS vs Tick borne illness given the extent of thrombocytopenia and subsequent upper extremity petechiae appearing a few days into the patient's admission. However ADAMTS13 and coagulation function were normal, our patient was shiga-toxin negative, and Rickettsia serology negative. The patient was found to have coagulase negative staphylococcus bacteremia which was successfully treated with 7 days of doxycycline. Bone marrow biopsy was notable for schistocytes suspicious for a low grade hemolytic process with no evidence of hematopoietic neoplasia or myelodysplasia. Platelets did not initially improve with blood transfusions remaining 30,0000-50,000. In concordance with the transfusions oral prednisone was given, however thrombocytopenia was refractory to this therapy as well. Lastly plasma exchanges were continued and Rituximab once weekly for three weeks was started; after the first dose our patients' platelets began to improve. Platelet count rose above goal of 150,000 and the patient was discharged with plan for once weekly Rituximab infusions for four weeks outpatient and oral Prednisone 40 mg daily.

Discussion:

ITP is usually found in females and is mostly asymptomatic. When symptoms appear they are characterized by things such as petechiae, purpura, gingival bleeding. The diagnosis is one of exclusion where patients present with thrombocytopenia and no other abnormalities. Treatment options for first line refractory ITP involve identifying and treating the underlying cause as well as interventions with thrombopoietin receptor agonists, Rituximab, and even splenectomy. These solutions can prove to be expensive and each provides their own risks. Cases such as this emphasize the importance of broadening differential diagnosis when presentation is not typical and initial treatments seem to be refractory.

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