ABSTRACT
Chronic myelomonocytic leukemia (CMML) presents as a complex hematologic malignancy with myelodysplastic and myeloproliferative features. Our case report explores the rare coexistence of CMML with immune thrombocytopenic purpura (ITP) in a 63-year-old female patient. CMML diagnosis followed World Health Organization criteria, and the patient was classified as having high-risk myelodysplastic syndrome (MDS)-CMML stage 2. Initial treatment with subcutaneous azacytidine for CMML proved partially effective, highlighting persistent severe thrombocytopenia. Subsequent investigations revealed secondary ITP associated with Crohn's disease. Conventional ITP therapies, including high-dose steroids and intravenous immunoglobulin, showed limited efficacy. Eltrombopag, a thrombopoietin receptor agonist, was initiated, resulting in the normalization of platelet counts within six weeks. Our case emphasizes the diagnostic challenges and intricate treatment landscape of CMML-associated ITP, suggesting eltrombopag as a potential therapeutic option in refractory cases. The study contributes to the evolving understanding of the complex interplay between myeloid disorders and immune-mediated hematological conditions, calling for personalized and multidisciplinary approaches to enhance patient outcomes.
ABSTRACT
INTRODUCTION AND IMPORTANCE: Early clinical presentations of spinal processes involving the epidural space are often vague and can mimic other spinal nerve impingements. Patients with NHLs frequently experience neurological problems due to metastatic spinal cord compression (MSCC). CASE PRESENTATION: In this case report, we present a 66-year-old female patient who was diagnosed with diffuse large B-cell lymphoma (DLCBL) of the sacral spine after a recurrence of cauda equine syndrome. The patient initially presented with back discomfort, radicular pain, and muscle weakness, which progressed to weakness in the lower extremities and bladder dysfunction over a few weeks. The patient was treated by surgical decompression and the biopsy result revealed a diagnosis of DLBCL. Further workup proved the tumor is primary and the patient as treated with radio- and chemotherapy. CLINICAL DISCUSSION: The varied distribution of symptoms based on the spinal level of the lesion makes early clinical diagnosis of spinal NHL challenging. In this case, the patient's initial symptoms closely resembled intervertebral disc herniation or other spinal nerve impingements, which delayed the diagnosis of NHL. The abrupt onset and progression of neurological symptoms in the lower extremities and bladder dysfunction raised the suspicion of MSCC. CONCLUSION: NHLs can present as metastatic spinal cord compression, which can cause neurological problems. Early clinical diagnosis of spinal NHLs is challenging due to the vague and varied presentations. A high index of suspicion for MSCC should be maintained in patients with NHLs who present with neurological symptoms.
ABSTRACT
Amyloidosis was initially described by Nicolaes Fonteyn in 1639. It is caused by the deposition of soluble immunoglobulin light chains as insoluble fibrils and can affect any organ including the heart, nervous system, dermis and subcutaneous tissue, kidneys and liver. This is a rare case of cardiac amyloidosis occurring with multiple myeloma and pleural effusion. A 65-year-old Arab woman, nonsmoker, nonalcoholic, known to have hypothyroidism and multiple myeloma, presented to the hospital with dyspnea and basilar crackles. Workup was done to exclude leukemia. Cardiac echography showed features of amyloidosis. Cardiac amyloidosis occurring with multiple myeloma and pleural effusion is rare. However, it is stated that 10-15% of amyloidosis patients might develop multiple myeloma (MM).
ABSTRACT
Purpose: We determine if monomethyl fumarate (MMF) can protect the retina in mice subjected to light-induced retinopathy (LIR). Methods: Albino BALB/c mice were intraperitoneally injected with 50 to 100 mg/kg MMF before or after exposure to bright white light (10,000 lux) for 1 hour. Seven days after light exposure, retinal structure and function were evaluated by optical coherence tomography (OCT) and electroretinography (ERG), respectively. Retinal histology also was performed to evaluate photoreceptor loss. Expression levels of Hcar2 and markers of microglia activation were measured by quantitative PCR (qPCR) in the neural retina with and without microglia depletion. At 24 hours after light exposure, retinal sections and whole mount retinas were stained with Iba1 to evaluate microglia status. The effect of MMF on the nuclear factor kB subunit 1 (NF-kB) and Nrf2 pathways was measured by qPCR and Western blot. Results: MMF administered before light exposure mediated dose-dependent neuroprotection in a mouse model of LIR. A single dose of 100 mg/kg MMF fully protected retinal structure and function without side effects. Expression of the Hcar2 receptor and the microglia marker Cd14 were upregulated by LIR, but suppressed by MMF. Depleting microglia reduced Hcar2 expression and its upregulation by LIR. Microglial activation, upregulation of proinflammatory genes (Nlrp3, Caspase1, Il-1ß, Tnf-α), and upregulation of antioxidative stress genes (Hmox1) associated with LIR were mitigated by MMF treatment. Conclusions: MMF can completely protect the retina from LIR in BALB/c mice. Expression of Hcar2, the receptor of MMF, is microglia-dependent in the neural retina. MMF-mediated neuroprotection was associated with attenuation of microglia activation, inflammation and oxidative stress in the retina.
