Aaron Niblock^1,2, Jerry Sin¹, Ciara Gore¹

¹Department of Haematology, Antrim Area Hospital, Northern Ireland

²School of Medicine, Ulster University, Northern Ireland

Corresponding Author: Aaron Niblock, Haematology department, Antrim Area Hospital, Northern Ireland; School of Medicine, Ulster University, Northern Ireland

Citation: Aaron Niblock, Jerry Sin, Ciara Gore (2023). The Utility of Immature Platelet Fraction Monitoring During a Case of Life-Threatening ITP Predicted Haemorrhagic Risk. World J Case Rep Clin Imag. 2023 October-November; 2(2)1-4.

Copyrights © 2023, Aaron Niblock, et al. This article is licensed under the Creative Commons Attribution-Non-Commercial-4.0-International-License-(CCBY-NC) (https://worldjournalofcasereports.org/blogpage/copyright-policy). Usage and distribution for commercial purposes require written permission.

Introduction

A severe case of immune thrombocytopenia purpura ITP presented with life threatening bleeding. Despite first line steroids and intravenous immunoglobulin IVIg this patient continued to deteriorate requiring full dose elthrombopag, romiplostim, cyclophosphamide, anti D, mycophenolate mofetil MMF, 31 units of platelets and 14 units of packed red cells. It took 3 weeks before the platelets responded, and the patient went on to make a full functional recovery despite severe pulmonary haemorrhages and a pneumothorax.

Immature platelet fraction IPF measurements are now more readily available given the ability of high-end full blood count analysers to perform them. A high IPF percentage would correlate with increased megakaryopoiesis in the bone marrow. When combined with a thrombocytopenia it would favour a consumptive process like ITP as the underlying aetiology.

In this case IPF was monitored concurrently with the absolute platelet count. Interestingly when the IPF fell during their admission the patient demonstrated overt bleeding. If the bleeding phenotype can be more accurately risk stratified this could help the clinician identify lower risk patients allow them to be managed in an outpatient setting. A larger study of ITP patients is required with IPF measurements to see if a robust risk stratification is possible.

Case Presentation

Mr. M presented to the Direct Assessment Area with bleeding gums and spontaneous bruising. He gave no history of a recent viral illness or any red flag symptoms, and no recent medications including vaccinations were administered.

On examination, he was clinically well and haemodynamically stable. He had a petechial rash over his lower extremities and areas of ecchymosis over his arms and torso.

Blood tests revealed a platelet count of 3 (x10⁹ /L) with a normal haemoglobin and white cell count. No haemolysis was evident, and haematinics were replete. After morphological examination of the blood film confirmed a true thrombocytopenia and a coagulation screen came back normal the working diagnosis was Immune Thrombocytopenia Purpura ITP.

Further investigations were performed including: a chest Xray; a viral screen- HIV, Hepatitis B and C, Cytomegalovirus, Epstein Barr Virus, H pylori and a CT chest, abdomen, pelvis all of which demonstrated no trigger for the ITP.

He was started on Prednisolone 1mg/kg (80mg) daily. Unfortunately, within several days frank haematuria occurred and as the platelets were still low, he received emergency intravenous immunoglobulins (IV Ig) 1g/kg for 2 consecutive days.

On the night after the IV Ig he developed a cough and haemoptysis. At the same time, melaena was evident and progressive ecchymosis. His haemoglobin dropped down to 67. Given this emergency multiple platelet transfusions were administered. Rituximab was infused and elthrombopag was started. A bone marrow biopsy was performed to exclude any other pathology driving the process but later was reported in keeping with ITP.

Mr M continued to deteriorate requiring packed red cells to maintain his haemoglobin and platelets as required. Oxygen requirements were increasing steadily, elthrombopag was increased to maximum dose as well as romiplostin. He reached the maximum oxygen that could be delivered on the ward therefore the risk/benefit approach was taken and cyclophosphamide with vincristine chemotherapy was administered. Interventional radiology was consulted for possible splenic embolisation and surgeons for consideration for an emergency splenectomy.