Gwenalyn Garcia

Antineoplastic agents and thrombotic microangiopathy

Thrombotic microangiopathy is an uncommon but reported adverse effect of a variety of antineoplastic drugs, including chemotherapy agents such as mitomycin C and gemcitabine, and newer targeted agents such as the vascular endothelial growth factor inhibitors. We present a review of thrombotic microangiopathy associated with antineoplastic agents and its implications in current cancer therapy.

Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient—A High Suspicion Is Still Needed

Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the activation of endothelial cells, monocytes, platelets, and complement by aPLs. Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present a case of a 34-year-old male referred for pancytopenia and splenomegaly. On examination, he had decreased sensation and 4/5 power in the left upper extremity. A lacy, purplish rash was noted on the trunk and upper extremity. MRI of brain showed acute/subacute lacunar infarctions. Laboratory studies revealed an elevated lactate dehydrogenase level, bilirubin and ferritin, decreased haptoglobin, and positive Coombs test. Antinuclear antibody test was negative and antiphospholipid antibody panel revealed positivity for anti-cardiolipin IgG and IgM, antiphosphatidylserine IgG, and anti-β2-glycoprotein IgG. The patient was diagnosed with primary APS. Pancytopenia is relatively rare in primary APS and is more often seen in secondary APS. Our patient demonstrated involvement of multiple organ systems as well as livedo reticularis and autoimmune-related findings such as Raynaud phenomenon and Coombs positive hemolytic anemia. We discuss the various clinical and laboratory findings in patients with APS that aid in diagnosis, as well as important management considerations.

Systemic Mastocytosis with Smoldering Multiple Myeloma: Report of a Case

Systemic mastocytosis (SM) is a disease characterized by a clonal infiltration of mast cells affecting various tissues of the body. It is grouped into six different subtypes according to the World Health Organization classification. It is called indolent systemic mastocytosis (ISM) when there is no evidence of end organ dysfunction, while the presence of end organ dysfunction defines aggressive systemic mastocytosis (ASM). When SM coexists with a clonal hematological disorder, it is classified as systemic mastocytosis with associated clonal hematological nonmast cell lineage disease (SM-AHNMD). Over 80% of SM-AHNMD cases involve disorders of the myeloid cell lines. To our knowledge, there are only 8 reported cases to date of SM associated with a plasma cell disorder. We report a patient with ISM who was found to have concomitant smoldering multiple myeloma. His disease later progressed to ASM. We discuss this rare association between SM and a plasma cell disorder, and potential common pathophysiologic mechanisms linking the two disorders will be reviewed. We also discuss prognostic factors in SM as well as the management options considered during the evolution of the patients disease.

Long-term Survival in a Patient with Recurrent Pulmonary Metastases from Uterine Leiomyosarcoma

Chemotherapy is the standard of care for disseminated uterine leiomyosarcoma; however, long-term survival is rarely achieved with this aggressive disease. We report a patient with recurrent pulmonary metastatic disease from uterine leiomyosarcoma who is doing well without significant disease nine years after her initial diagnosis. We discuss her clinical course, the treatment modalities she received, and the clinical evidence supporting these.

Calreticulin Mutated Essential Thrombocythemia Presenting as Acute Coronary Syndrome

Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by a clonal expansion of megakaryocytes. ET can result in both arterial and venous thrombosis. Involvement of the coronary arteries has been reported. Patients who harbor a CALR mutation are half as likely to suffer a thrombotic event as compared to patients with a JAK2 mutation. We report a case of CALR-mutated ET whose initial disease manifestation was a non-ST segment elevation myocardial infarction.

Synchronous Pulmonary Neoplasms: A Chance Occurrence or is There More Than Meets the Eye?

Primary bronchus-associated lymphoid tissue (BALT) lymphoma comprises 5% of non-Hodgkin’s lymphoma (NHL) and usually has an indolent course. Synchronous primary lung cancers with BALT lymphoma are seldom seen in patients with adenocarcinoma of the lung. Synchronous squamous cell carcinoma (SCC) and BALT lymphoma is an extremely rare occurrence. We report an unusual case of stage 4 BALT lymphoma requiring treatment that revealed an underlying ipsilateral mass causing a diagnostic dilemma. An 84-year-old female with a history of systemic lupus erythematosus, deep vein thrombosis, and thrombotic microangiopathy presented to the hospital with cough and dyspnea on exertion. A chest X-ray revealed right hemi-thorax opacification and computed tomography (CT) of the chest showed a large right effusion and a soft tissue density extending into the proximal right bronchus. She required repeated thoracentesis until the pleural fluid analysis showed the presence of small lymphocytes and bronchial washings revealed an abnormal B cell population consistent with extranodal marginal zone lymphoma. The patient received four cycles of bendamustine and rituximab resulting in near-complete resolution of the effusion. Four months from diagnosis, imaging showed an increase in the size of the soft tissue density with pathologic fluorodeoxyglucose (FDG) uptake on positron emission tomography (PET). A CT-guided biopsy was consistent with squamous cell lung cancer (SCLC) and radiotherapy was started for clinical stage 2 disease since the patient was not a surgical candidate. BALT lymphoma is a low-grade malignancy classified as extranodal marginal zone lymphoma with a five-year survival rate of over 80%. Several cases of synchronous lung adenocarcinoma and BALT lymphoma have been described. However, our case is among the rare few cases of synchronous occurrence of SCLC with BALT lymphoma. This report highlights the challenges associated with establishing an accurate and timely diagnosis.

Systemic Combination Chemotherapy in Elderly Pancreatic Cancer: a Review

PurposeIn recent years, significant progress in survival has been achieved using systemic combination chemotherapy in patients with pancreatic cancer. However, the elderly are largely underrepresented in clinical trials, and gains made from these may not necessarily apply to this important subgroup of patients. We review the currently available data regarding contemporary combination chemotherapy regimens, including FOLFIRINOX, gemcitabine plus nab-paclitaxel, nanoliposomal irinotecan plus 5-fluorouracil and leucovorin, and gemcitabine plus capecitabine, in elderly pancreatic cancer patients.MethodsWe performed a search of Pubmed using the terms “pancreatic cancer”, “elderly”, “FOLFIRINOX”, “gemcitabine”, “nab-paclitaxel”, “capecitabine”, and “nanoliposomal irinotecan” and included articles investigating the use of combination chemotherapy in the elderly with pancreatic adenocarcinoma. Relevant abstracts from American Society of Clinical Oncology and European Society of Medical Oncology meetings were included.ResultsCurrent clinical evidence and experience suggests that relatively fit elderly pancreatic cancer patients may derive significant benefit from contemporary combination chemotherapy regimens. Strategies to improve tolerability without decreasing efficacy include dose reduction, schedule modification, and growth factor support. Phase III clinical trials are ongoing to determine the optimal use of combination chemotherapy regimens in elderly patients with pancreatic cancer.ConclusionIdentifying elderly patients who will benefit from combination chemotherapy for pancreatic cancer remains a significant clinical challenge. An assessment of medical comorbidities and functional status plays a key role in determining fitness for intensive chemotherapeutic regimens in this important subset of patients.

Adenocarcinoma Ex-goblet Cell Carcinoid of the Appendix: a Case Report and Overview of the Disease

Goblet cell carcinoid (GCC) of the appendix is a rare disease with varying guidelines for histopathologic diagnosis and management. The disease is intriguing in terms of its pathologic features. Three subtypes have been described depending on the presence of signet ring cells and extent of differentiation of the adenocarcinoma component [1]. The clinical behavior of the tumor ranges from aggressive to relatively indolent disease. Treatment is based on extent of the disease, grade of the tumor, and individual patient characteristics. Whereas surgical resection remains the mainstay of resectable disease, adjuvant chemotherapy and intraperitoneal chemotherapy have been employed. We discuss our experience with diagnosis and management of the disease in a patient we encountered.

Composite Diffuse Large B-cell and Mantle Cell Lymphoma: A Case Report

Composite lymphoma is an extremely rare clinical entity and is characterized by the presence of two different subtypes of lymphoma in the same lymph node. We report a case of composite lymphoma in a 57-year-old male presenting with leg and groin pain. The right inguinal lymph node biopsy showed large and small cells. Immunohistochemistry was consistent with large cells staining for diffuse large B-cell lymphoma (DLBCL) and small cells positive for mantle cell lymphoma (MCL). Polymerase chain reaction (PCR) analysis of immunoglobulin heavy (IGH) chain and immunoglobulin kappa (IGK) light chain gene rearrangements confirmed that the two were clonally unrelated neoplasms. There are only two reported cases of composite lymphoma with this combination in the published English literature. We report the third such case and discuss the pathology, diagnostic challenges and management of composite lymphoma.

T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease

T cell histiocyte rich large B cell lymphoma (THRLBCL) is a rare subtype of non-Hodgkins lymphoma characterized by malignant B cells with reactive T lymphocytes. The pathophysiology is thought to involve cytokine-mediated evasion of T cell immune response by malignant B cells. It usually presents at an advanced stage with extranodal involvement. An extremely unusual manifestation of the disease is hemophagocytic lymphohistiocytosis (HLH) which is a hyperinflammatory disorder. We present a case of a 43-year-old male who presented with recurrent fever and recent radiologic imaging showing splenomegaly and right inguinal lymphadenopathy. On presentation, he had a fever of 105°F. Laboratory work-up was consistent with pancytopenia, elevated lactate dehydrogenase, elevated D-dimer, and a ferritin of 24,247 ng/mL. The patient was started on steroid therapy. An excisional biopsy of the right inguinal lymph node was consistent with a diagnosis of THRLBCL and the patient subsequently received six cycles of chemotherapy with R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) after which a PET-CT scan showed no evidence of biologically active disease and ferritin was down to 822 ng/mL. We discuss the clinical manifestations and diagnostic and therapeutic considerations of this rare disease along with a review of reported cases in the literature.