63-year-old male with past medical history of ulcerative colitis s/p colectomy with ileal reservoir in childhood, hypertension, hyperlipidemia, and type 2 diabetes mellitus was originally diagnosed with ITP in September 2010. Initial treatment was with pulse dose dexamethasone and IVIG with transient response; platelet count remained in single digits. Additionally, there was no response noted when patient was treated empirically for Helicobacter Pylori. In November 2010, patient was treated with Rituximab, and the platelet count became normal.
Patient had a relapse of ITP in October 2012 and underwent laparoscopic splenectomy in November 2012. Platelet count improved transiently but relapsed again in February 2013. Bone marrow biopsy was performed at the time and showed megakaryocytic hyperplasia. Also, flow cytometry showed increased T cells at about 10% consistent with large granular lymphocytic leukemia (LGL). T cell gene rearrangement studies were positive by PCR. Eltrombopag was started in April 2013 and platelet count became normal. This medication was gradually tapered and stopped at the end of 2013. Platelet count remained normal.
Unfortunately, patient was diagnosed with autoimmune hemolytic anemia in May 2014 and was given one dose of Cyclophosphamide and four doses of Rituximab. The platelet count was still within normal limits. In June 2014, patient was diagnosed with bilateral pulmonary embolism and left lower extremity deep vein thrombosis. He was treated with Enoxaparin and Warfarin. Despite this and developing recurrent MRSA infections as well as endocarditis, platelet count remained in the normal range for the next few years.
Patient had a relapse of ITP in November 2017 with platelets of 1,000 per microliter of blood and spontaneous bleeding; he received IVIG as well as steroids without response. Additionally, he was given Romiplostim from December 2017 to September 2018 and Rituximab from February 2018 to March 2018, resulting in normalization of platelet count. However, patient had another relapse in July 2020 and Eltrombopag was given from July 2020-September 2021, which resulted in normalization of platelet count.
In June 2024, patient relapsed of ITP once more with platelet count <3,000 per microliter of blood. During this hospitalization, patient was given IVIG and pulse dose Dexamethasone twice followed by Romiplostim. Later, he was given Eltrombopag and discharged home with a platelet count of 18,000 per microliter of blood. However, in July 2024, patient was readmitted with platelet count of 3,000 per microliter per blood. Repeat bone marrow biopsy again showed megakaryocytic hyperplasia and no evidence of malignancy (Figure 1). During this hospitalization, patient received multiple treatments in succession, namely four doses of pulse dose Dexamethsone, four weekly doses of subcutaneous Daratumumab, four doses of intravenous Rituximab, and one dose of subcutaneous Romiplostim along with oral Fostamatinib as the platelet count remained low (><=3x10^3/μL). Additionally, patient received multiple single donor platelet transfusions during this time. There was consideration for transfusing HLA-matched platelets, however, patient tested negative for HLA-antibodies.
However, in July 2024, patient was readmitted with platelet count of 3,000 per microliter per blood. Repeat bone marrow biopsy again showed megakaryocytic hyperplasia and no evidence of malignancy (Figure 1). During this hospitalization, patient received multiple treatments in succession, namely four doses of pulse dose Dexamethsone, four weekly doses of subcutaneous Daratumumab, four doses of intravenous Rituximab, and one dose of subcutaneous Romiplostim along with oral Fostamatinib as the platelet count remained low (<=3x10^3/μL). Additionally, patient received multiple single donor platelet transfusions during this time. There was consideration for transfusing HLA-matched platelets, however, patient tested negative for HLA-antibodies.
After all these interventions, platelet count started rising in midAugust and normalized by end of August 2024. Hospitalization was complicated by Kock pouch obstruction secondary to adhesions. Therefore, patient underwent exploratory laparotomy, small bowel resection x2, removal of Kock pouch, lysis of adhesions, and repair of peristomal hernia and incisional hernia in late August 2024. Patient was discharged on oral Fostamatinib in September 2024. Since then, platelet count remains normal to date.
