Chronic Myelomonocytic Leukemia-Associated Immune Thrombocytopenic Purpura: A Report of a Rare Case and a Review of Literature.

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.

Neoadjuvant Chemotherapy Approach to Pineal Germinoma: A Case Report.

Intracranial germ cell tumors (GCTs) are rare malignant tumors with a peak incidence around puberty. The pineal region is the most commonly involved area of all intracranial GCTs. Due to the heterogeneous tumor origin, subtypes, and presentation, diagnosis and management are challenging. Complicated pineal germinomas are rarely reported in the literature. Here, we report a rare case of pineal germinoma with hydrocephalus and discuss the potential treatment approach. A 20-year-old boy presented to the hospital with vomiting and a decreased level of consciousness. The brain magnetic resonance imaging (MRI) revealed a pineal tumor. A ventriculoperitoneal shunt was placed to relieve the increased intracranial pressure. The patient underwent a suboccipital craniotomy with excisional biopsy of the pineal region tumor due to its critical location, as imaging studies alone may not be sufficient to establish a definitive diagnosis. Although there has been a rise in reported cases of germinoma tumors, there is currently no standardized therapeutic approach for treating them. Therefore, more randomized controlled cohort studies are necessary to evaluate potential treatments and develop a therapeutic approach.

Navigating treatment options for sacral spine lymphoma: A medical journey.

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.

Atypical Bullous Pemphigoid After Linagliptin Intake.

BACKGROUND Bullous pemphigoid is a common pruritic skin lesion reported in elderly patients. It is caused by an immunologic reaction between autoantibodies and hemidesmosome proteins of epithelial cells. The disease is characterized by a symmetrical blister distribution on the body. Diagnosis should be suspected in elderly patients presenting with a tense blister on normal-appearing skin or on an erythematous base. In the literature, several forms of typical bullous pemphigoid after treatment with linagliptin have been reported. However, this is the first reported case of atypical nonbullous pemphigoid after linagliptin intake. CASE REPORT A 77-year-old woman presented with multiple erythematous papules and nodules on the upper extremities and trunk. The patient was being treated with linagliptin for diabetes. Diagnosis was made with biopsy and histopathological studies, followed by direct immunofluorescence. The histopathological study showed a subepidermal blister with an underlying polymorphous infiltrate, mainly of an eosinophilic profile. Direct immunofluorescence showed linear IgG and C3 antibodies to hemidesmosomes at the lamina lucida of the basement membrane. Thus, the diagnosis of atypical nonbullous pemphigoid was made. CONCLUSIONS This report emphasizes the great variety of bullous pemphigoid presentation and the need for a greater level of awareness of the adverse effects of linagliptin. Thus, atypical nonbullous pemphigoid should be considered among the potential differential diagnoses in patients with multiple erythematous papules and nodules on the upper extremities and trunk.

Cardiac amyloidosis with overt multiple myeloma presenting with pulmonary effusion: case report.

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).

The role of 18F-FDG PET/CT scan compared to CT-scan alone for lymph node staging before radical cystectomy in patients with bladder cancer.

BACKGROUND: Accurate Lymph node (LN) staging before radical cystectomy (RC) in patients with bladder cancer (BC) is crucial to improve patient's management. 18F-fluorodeoxyglucose positron-emission tomography-computed tomography (FDG-PET-CT) become widely used in the loco-regional staging of BC. The diagnostic performance of PET-CT in preoperative LN staging of BC is still unknown due to lacking large trials. OBJECTIVES: We aim to evaluate the diagnostic value of PET-CT scan, compared with CT scan alone for preoperative LN staging of BC. PATIENTS AND METHODS: From January 2010 to November 2020, we retrospectively reviewed the records of 300 patients undergoing RC for muscle-invasive BC and high-risk non-muscle-invasive BC. All patients had PET-CT and CT of abdomen and pelvis to assess for pelvic LN metastases before RC. Patients were excluded from analysis if they had neoadjuvant chemotherapy (NAC). Sensitivity, specificity, and accuracy for detecting pelvic LN metastases were determined by comparing the results of the FDG PET-CT and CT alone to the final histopathology reports obtained after RC. RESULTS: LN metastasis was confirmed histology in 134 patients (44.7%). On a patient-based analysis, PET-CT, and CT showed a sensitivity of 40.3% and 13.4 %, respectively, a specificity of 79.5% and 86.7 %, respectively, positive predictive value (PPV) of 61.4% and 45%, respectively, and negative predictive value (NPV) of 62.3% and 55.4%, respectively. The diagnostic accuracy of PET-CT scan depends on multiple preoperative and postoperative factors. CONCLUSION: PET-CT is more accurate than CT-scan alone for preoperative LN staging in patients with BC.

The First Reported Case of Neurotrophic Tyrosine Receptor Kinase Fusion-Positive Thymoma Treated Successfully With Entrectinib.

We present the first reported case of stage 4 thymoma with pleural metastases that was found to be driven by the neurotrophic tyrosine receptor kinase (NTRK)-fusion gene. The patient was started on chemotherapy but it was discontinued due to intolerable side effects. Alternative options in such patients with rare diseases are limited; in fact, many concerns exist regarding the safety and efficacy of newly approved agents for the treatment of advanced thymomas, such as pembrolizumab and sunitinib. Due to NTRK-fusion gene positivity, entrectinib, a novel NTRK-fusion inhibitor, was then initiated. This drug has shown an objective response of 57% in treating NTRK fusion-positive solid tumors of 19 different histological subtypes, predominantly sarcomas, non-small cell lung cancer (NSCLC), and mammary analogue secretory carcinoma of the salivary gland. However, it has never been assessed in the treatment of thymomas. After 10 months of follow-up, the patient showed a significant response with mild adverse events, which was managed by temporary discontinuation of the drug. This case highlights the crucial role of whole-genome sequencing and tissue-agnostic antineoplastics in the future of cancer treatment.

Novel anticancer therapy in BCG unresponsive non-muscle-invasive bladder cancer.

INTRODUCTION: Many patients with non-muscle-invasive bladder cancer (NMIBC) failed intravesical BCG therapy. Currently, radical cystectomy is the recommended standard of care for those patients. There is unfortunately no effective other second-line therapy recommended. AREAS COVERED: In this review, we present the topics of BCG unresponsive NMIBC; definition, prognosis, and further treatment options: immunotherapy, intravesical chemotherapy, gene therapy, and targeted individualized therapy. EXPERT OPINION: There are major challenges of the management of NMIBC who failed BCG therapy as many patients refuse or are unfit for radical cystectomy. Multiple new modalities currently under investigation in ongoing clinical trials to better treat this category of patients. Immunotherapy, especially PD-1/PD-L1 inhibitors, offers exciting and potentially effective strategies for the treatment of BCG unresponsive NMIBC. As the data expands, it is sure that soon there will be established new guidelines for NMIBC.

Inguinal lymph node metastasis of bladder carcinoma after radical cystectomy: A case report and review of literature.

INTRODUCTION: Lymph node metastasis in bladder cancer (BC) is common and has been associated with a very poor prognosis. Bc rarely metastasizes to inguinal lymph nodes. PRESENTATION OF CASE: We reported an unusual case of right inguinal lymph node metastasis of transitional cell carcinoma of the bladder. Metastasis occurred 9 months after radical cystectomy for BC. The patient refused chemotherapy and underwent only surgical excision of lymph nodes without any adjuvant therapy. During a follow-up period of 3 years, the patient still having complete remission. DISCUSSION: Multiple studies showed an improved clinical outcome with adjuvant chemotherapy for pathological node-positive patients with BC. Long-term survival could be achieved for some patients with limited lymph node metastasis who underwent metastasectomy. Some studies supported the benefit of surgical consolidation after a good response to systemic chemotherapy. The best management plan for clinically node-positive BC is not established yet. CONCLUSION: There is little evidence on which to base the management of inguinal lymph node metastasis from BC. Metastasectomy could be an option with good outcomes.