[BILATERAL OCULAR PLASMACYTOMA PRESENTING AS ACUTE ANTERIOR UVEITIS].

INTRODUCTION: A 64-year-old woman, presented to the ophthalmology outpatient clinic with abrupt onset of pain, blurred vision and redness in her right eye. Her medical history is remarkable for multiple myeloma, with successful bone marrow transplantation and recurrent disease as secondary plasma cell leukemia, managed with a biologic agent. Examination revealed severe fibrinous anterior uveitis, accompanied by keratic precipitates, rubeosis iridis and raised intra-ocular pressure (IOP) and normal fundus. The patient was treated intensively with topical steroids, IOP lowering agents, systemic acyclovir and prednisone. Response was partial, with further development of posterior synechiae, iris bombe and 360o angle closure. An anterior segment ultrasound demonstrated severely thickened iris. The appearance of bilateral sub-conjunctival salmon patches in both eyes facilitated histopathologic diagnosis by extraocular tissue biopsy which revealed plasma cells infiltration, suggestive of plasmacytoma. Ruthenium plaque radiotherapy administered in a 40Gy dose was initiated in both eyes with a new cycle of systemic chemotherapy. Complete regression after treatment was achieved bilaterally. AIMS: To report a case of bilateral ocular plasmacytoma presenting as an acute anterior uveitis. BACKGROUND: Plasmacytoma, associated with multiple myeloma, is a solid tumor of the lymphoid system composed of monoclonal plasma cells. Ocular involvement is rare and direct infiltration of both eyes is even rarer. METHODS: A case report. CONCLUSIONS: Extramedullary plasmacytoma rarely involves ocular structures. In the setting of systemic malignancy, ocular involvement should be considered, especially when acute inflammation is recalcitrant to therapy.

T-Lymphoblastic Leukemia/Lymphoma and Thymoma: A Case Report and Review of the Literature of a Rare Association.

The co-occurrence of thymoma and T-lymphoblastic lymphoma/leukemia is an extremely rare but previously reported association that poses a diagnostic and therapeutic challenge. We describe a 67-year-old patient with long-standing untreated B1 thymoma that presented with constitutional symptoms and a painless soft tissue mass on the right chest wall. Pathological analysis of the biopsy from the mass demonstrated T-lymphoblastic leukemia/lymphoma. The patient went through a complicated course, was refractory to several lines of therapy, and eventually underwent allogeneic hematopoietic stem cell transplantation in complete remission from a matched related donor. The association between thymoma and malignant neoplasms has been described in the literature, most notably with colorectal adenocarcinoma and thyroid cancer. Thymoma-associated leukemia is, however, extremely unusual, with limited reports in the literature. Distinguishing between thymoma and leukemia can be challenging and often requires meticulous diagnostic efforts. For patients with a past history of thymoma, awareness of this particular association should be bared in mind to allow earlier diagnosis and therapy.

Autoimmune and inflammatory manifestations associated with acute myeloid leukemia with Trisomy 8-Case series and review of the literature.

AML can be associated with autoimmune or inflammatory phenomena (AIP) occurring prior, concomitantly, or after its diagnosis. Trisomy 8 is one of the most common cytogenetic abnormalities associated with AML. We describe three patients with AML, trisomy 8, and associated AIP and review the known literature on this association. All of our patients had major symptomatic relief when treated with leukemia-directed therapy and corticosteroids. AIP in AML may be an underdiagnosed phenomenon, particularly in patients with trisomy 8.

Skin biopsies in acute myeloid leukemia patients undergoing intensive chemotherapy are safe and effect patient management.

There is paucity of data regarding the diagnostic yield and safety of skin biopsies in patients with acute myeloid leukemia (AML), though skin eruptions are common in these patients. We evaluated 216 patients treated in our hemato-oncology unit at a tertiary medical center between 2007 and 2018 and identified 35 patients who underwent 37 skin biopsies. The majority of biopsies were performed during induction treatment for AML (n = 26, 70%), whereas the remainder of biopsies were done prior to induction initiation (n = 8, 22%) or during consolidation chemotherapy (n = 3, 8%). Pathology findings were inconclusive in 13 cases (35%), while diagnostic biopsies were positive for drug eruptions (24%), leukemia cutis (16%), infections (11%), reactive processes (8%) and Sweet syndrome (5.5%). In almost half of cases (16/37) tissue cultures were performed. Of those, only a quarter (4/16) were positive. Histopathology and tissue culture results altered immediate patient care in 3 cases (8%), yet information obtained from biopsies had potential to affect long term patient care in 8 additional cases (21.6%). Although most skin biopsies were performed while patients had severe thrombocytopenia and neutropenia, only one patient had a complication due to the biopsy (fever and local bleeding). With the limitation of a retrospective analysis, our study suggests that skin biopsies in patients treated for AML are relatively safe. Although biopsy results infrequently alter immediate patient management, long term effect on patient care expand the potential diagnostic yield of skin biopsies.

Leukemic Phase of Histiocytic Sarcoma of the Digestive System: A Rare Manifestation of a Rare Disease.

Histiocytic sarcoma (HS) is a rare, malignant, and aggressive subtype of histiocytosis. We present an unusual case of aggressive HS presenting in the gastrointestinal tract and gallbladder that progressed after several lines of chemotherapy with a leukemic phase. We review the clinical, pathological, and molecular characteristics of HS in this case and review the literature on HS involving the digestive system as well as on overt leukemic phase of this disease. HS is often diagnosed at an advanced stage, and mortality is high. We discuss the therapeutic approach to patients with HS. We highlight the role of overexpression and somatic alterations in the RAF-MEK-ERK pathway in the pathogenesis of HS and discuss potential targeted approaches to treat these rare tumors.

Hemophagocytic lymphohistiocytosis as a harbinger of aggressive lymphoma: a case series.

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome, which can manifest either secondary to a variety of underlying causes, or due to a primary genetic defect. Malignancy is the most common underlying disease in adults with HLH, with lymphomas being the most common malignancy. Lymphoma-associated hemophagocytic syndrome (LAHS) typically follows a rapidly progressive clinical course and is associated with poor prognosis. We herein present four patients with HLH associated with aggressive lymphoma. At initial presentation, the underlying etiology of the HLH was unclear. Two patients were eventually diagnosed with anaplastic large cell lymphoma, while the other two had diffuse large B cell lymphoma. Two of the patients experienced rapid clinical deterioration, one at diagnosis and the other at relapse, and both died prior to diagnosis of lymphoma despite HLH-directed therapy. These cases highlight the need for intensive management in adults with HLH without a clear etiology, especially in cases when lymphoma-associated HLH is suspected. We describe the current pitfalls in diagnosis and treatment of LAHS and discuss possible ways to improve patient management.

Spontaneous Regression of Hodgkin Lymphoma: Case Report and Review of the Literature.

Spontaneous regression of Hodgkin lymphoma (HL) is a rare event. We describe a 32-year-old woman with spontaneous regression of HL and review the literature. The patient presented with cervical lymphadenopathy and was diagnosed with stage IIA classical HL. The patient refused to receive any treatment for her disease. Positron emission tomography/computed tomography carried out 2 years later showed complete regression of the lymphadenopathy, without pathological uptake of fluorodeoxyglucose. At the last follow-up, 3.5 years after the initial presentation, the patient is with no evidence of disease. During workup for the HL, concomitant papillary thyroid carcinoma was diagnosed, for which the patient refused treatment as well. The thyroid malignancy has remained stable throughout the follow-up.

Agranulocytosis Associated with Waldenström Macroglobulinemia.

Currently, there are only 2 case reports of Waldenström macroglobulinemia (WM) associated with severe neutropenia. This is a case report of a woman with a past medical history of WM who presented with neutropenic fever. The patient's febrile neutropenia resolved after RCD chemotherapy (cyclophosphamide 750 mg/m2, dexamethasone 20 mg, and rituximab 375 mg/m2). Fourteen days after administration, the neutrophil level had started to rise and normalized after 6 days. To the best of our knowledge, this is the 3rd reported case of agranulocytosis due to WM.