Ocular Manifestations in Hematological Disorders.

Background Ophthalmic manifestations are a common feature in hematological malignancies and may be divided into two groups: those attributable to the disease's infiltration of the eye, and the ocular consequences due to blood abnormalities. This study aims to determine the prevalence of ocular manifestations and their association with the different hematological disease subgroups. Materials and methods We conducted a retrospective and observational study enrolling 137 consecutive patients on active treatment or follow-up for hematological malignancies from January 2016 to January 2020 at the Cheikh Khalifa International University Hospital. All patients underwent a standardized and comprehensive ophthalmic evaluation. Results Ocular involvement was primarily disclosed in acute myeloid leukemia (AML), non-Hodgkin's lymphoma (NHL), and multiple myeloma (MM). We herein report the different ocular changes divided into anterior segment manifestations, posterior segment manifestations, dry eye disease, and ocular adnexa findings. Patients with leukemia had a significantly higher rate of lesions in the ocular posterior segment (p < 0.001). Despite the high prevalence of anterior segment and ocular adnexa findings in lymphoma patients, no significant association emerged between these lesions and the aforementioned condition. In addition, dry eye disease was found in all instances without any association with the disease itself. Conclusions Awareness of ocular pathology in hematological malignancies is important as it may precede systemic diagnosis or be a sign of recurrence. We should also be concerned about the side effects of treatments, predominantly, dry eye disease. Therefore, periodic ophthalmic assessment throughout the disease's course, as well as interdisciplinary coordination of care, is crucial to promote early diagnosis and treatment, hence improving long-term outcomes.

Optic Disc Drusen (ODD), an Often Misdiagnosed Disease: A Case Report.

Optic disc drusen (ODD) are abnormal collections of protein and calcium that accumulate within the optic nerve. We report a case of a 17-year-old girl who presented to the Department of Ophthalmology at the Cheikh Khalifa International University Hospital, Casablanca, Morocco, with a decline in visual acuity, visual field deficiency, and color vision abnormalities. The patient was misdiagnosed and mistreated for optic neuritis given the presence of bilateral Stage III papilledema solely. After many months of diagnostic wandering, a fundus examination revealed a white atrophic papilla with calcified deposits grouped in a crown around the papillary excavation, suggesting papillary drusen. When the deposits are visible on ophthalmoscopy and manifest as an elevation and a blurring of the optic disc's margins, their diagnosis remains straightforward. However, their identification might be problematic when they are firmly lodged in the optic disc or with the presence of papilledema, leading to confusion with other differential diagnoses, particularly if the condition affects both eyes. The purpose of this case report is to increase neurologists' and ophthalmologists' knowledge of the incidence of drusen in order to prevent excessive biological and imaging investigation in addition to harmful effects from needless drugs.

Acute Myeloid Leukemia Revealed by a Palatal Necrosis: A Rare Case Report.

Acute myeloid leukemia (AML) is an aggressive hematological malignancy due to genetic alterations characterized by an overproduction of neoplastic clonal myeloid stem cells in both the bone marrow and peripheral blood. We report a case of a 43-year-old man referred to the department of hematology with a three-week history of palatal pain and weakness. The physical examination revealed an ecchymosis on the left hard palatal mucosa and necrosis. The maxillofacial computerized tomography (CT) scan revealed large osteolysis of the left maxillary bone and a fistulated soft palate. The lesion's biopsy showed an acute polymorphic inflammation with no sign of malignancy. Laboratory findings revealed anemia, thrombocytopenia, elevated lactic dehydrogenase, and elevated serum ferritin. The diagnosis was subsequently confirmed by a peripheral-blood smear revealing 60% of circulating blasts and bone marrow aspiration with 80% of blast infiltration. The latter was further classified through cytogenetic studies as an AML with deletion of chromosome 7q. This case report aims to highlight the need for clinicians to be aware of palatal necrosis as an initial manifestation of the disease and to emphasize the role of multidisciplinary collaboration between dental surgeons, oral and maxillofacial surgeons, and hematologists for early detection and treatment.

Primary Plasma Cell Leukemia Revealed by a Mandibular Lesion: A Case Report.

Primary plasma cell leukemia (PCL) is a rare and aggressive hematological malignancy exhibiting a circulating plasma cell count exceeding 20% of peripheral blood leukocytes or an absolute plasma cell count >2000/mm3. We report a case of a 37-year-old woman presented to the Department of Hematology with a two-month history of growth inside the oral cavity in the upper jaw and weakness. The physical examination revealed a voluminous mass involving the left side of the maxillary gingiva. The maxillofacial computerized tomography (CT) scan confirmed the presence of a solid tissue mass at the left upper maxilla. A biopsy sample obtained from the lesion showed a plasma cell infiltration. The laboratory findings revealed anemia, renal impairment with high levels of creatinine and calcium. Serum protein electrophoresis found a monoclonal peak at IgG lambda, a high level of lambda free light. The diagnosis was subsequently confirmed by a peripheral-blood smear revealed 25% of plasma cells and bone marrow aspiration with 50% of plasma cell infiltration. Primary plasma-cell leukemia (pPCL) was confirmed. The patient received VTD chemotherapy (bortezomib, thalidomide, and dexamethasone) followed by autologous stem cell transplant (ASCT), which resulted in complete remission. At the six-month follow-up, the patient relapsed with extramedullary multiple lesions under ineffective rescue therapy. Response to frontline treatments may be significant initially but short-lived with a dismal median overall survival below one year. This case report aims to highlight the need for awareness among clinicians of the relevance of examining other associated clinical features of pPCL, given its aggressive course and rapid progress without the therapy.