Case Presentation: A 27-year old African American male presented with 3 months of bilateral inguinal lymph node enlargement, fevers, night sweats, fatigue, and weight loss. Patient presented for outpatient nodal biopsy and subsequently sent to the ED with severe thrombocytopenia with platelets less than 10,000 and complaints of gum bleeding and petechiae of 3 days. Patient remained thrombocytopenic despite platelet transfusions. Initial workup was negative for TTP, HUS, or DIC and he was diagnosed with ITP. Work-up included core needle biopsy of right lymph node and bone marrow aspirate; he was then started on IVIG and high dose steroids without improvement. While awaiting biopsy results, he developed blurry vision with floaters. Ophthalmology was consulted and he was diagnosed with intra-retinal hemorrhages attributed to severe anemia and thrombocytopenia. Anemia was attributed to extensive mucosal bleeding and purpura which was treated with tranexamic acid. Initial core-needle biopsies were concerning for Hodgkin’s but were non-diagnostic; steroids were held and excisional biopsy of left inguinal lymph node was obtained after which ITP treatment was restarted. He was diagnosed with classical Hodgkin’s lymphoma and started on ABVD chemotherapy with good response, however patient remained thrombocytopenic with refractory ITP. Thrombocytopenia finally resolved after splenectomy after several weeks of showing no response with steroids, IVIG, Romiplastim, and Rituximab. Vision remained unchanged at discharge.
Discussion: It is extremely rare for idiopathic thrombocytopenic purpura, or ITP to be associated with classical Hodgkin’s lymphoma and even more so for this to cause intra-retinal hemorrhages. Up until now, there have been less than 100 reported cases of ITP associated with classical Hodgkin’s lymphoma and less than 10 reported cases of retinal hemorrhage associated with ITP. There have been no reported cases with the combined association. We report the first case of the combined presentation. ITP is a rare complication for Hodgkin’s, but intra-retinal hemorrhages occurring as a subsequent complication to ITP and severe anemia has never before been reported together despite anemia being a known risk factor for developing retinal bleeding. Management of each can prove quite difficult. Treatment for ITP in Hodgkin’s is often unsuccessful even with first line and second line therapies and in many reported cases required splenectomy to fully resolve similar to this case. The management of retinal hemorrhage is extremely limited as there are no prescribed treatments or interventions.
Conclusions: This case illustrates the difficulty with any delay to final diagnosis as core needle biopsy proved insufficient and highlights timely diagnosis being extremely significant. It supports approaching suspected lymphoma with excisional biopsy first to avoid rare but devastating consequences. Further this case highlights the possibility of advancing to splenectomy for ITP sooner in patients who have evidence of bleeding or severe anemia. It should be noted that the literature commented that severe hemorrhage only occurred in those with concurrent severe anemia and thrombocytopenia. Whether prophylactic transfusions of blood and platelets would prevent these are unknown, but more research would help to know whether these complications are potentially preventable.