Faizan Malik

Guillain-Barré syndrome occurring synchronously with systemic lupus erythematosus as initial manifestation treated successfully with low-dose cyclophosphamide.

Systemic lupus erythematous (SLE) is frequently encountered in clinical practice; a widespread immunological response can involve any organ system, sometimes leading to rare and diagnostically challenging presentations. We describe a 38-year-old female who presented with symmetric numbness and tingling of the hands and feet, and cervical pain. Imaging studies were not diagnostic of any serious underlying pathology. The patient developed ascending paresis involving lower extremities and cranial muscles (dysphagia and facial weakness). Guillain–Barré syndrome (GBS) was diagnosed on the basis of electromyography and lumbar puncture showing albuminocytologic dissociation. Intravenous immunoglobulins (IVIG) were administered for 5 days. Supported by anti-dsDNA antibody, oral ulcers, proteinuria of 0.7 g in 24 h, and neurological manifestation, she was diagnosed with lupus. After completion of IVIG, she received pulse-dose corticosteroids and one dose of low-dose cyclophosphamide. Her neurological symptoms improved and she had complete neurological recovery several months after her initial presentation. Literature search provides evidence of co-occurrence of lupus and GBS occurring mostly later in the course of the disease. However, GBS as initial manifestation of SLE is exceedingly rare and less understood. The association of GBS with lupus is important to recognize for rapid initiation of appropriate therapy and for consideration of immunosuppressive therapy which may affect the outcome.

The tell-tale heart: A case of recurrent vulvar carcinoma with cardiac metastasis and review of literature

A 50-year-old female was diagnosed with vulvar cancer treated with left partial vulvectomy and bilateral lymphadenectomy. Ten months after her surgery, she presented with increased labial swelling, pain and discharge. Biopsy confirmed recurrence of squamous cell vulvar carcinoma. Incidentally, on restaging radiographic scans, she was found to have a large right ventricular mass which, after surgical debulking, was shown to be a squamous cell cancer of vulvar origin. She was commenced on chemotherapy with carboplatin and paclitaxel along with concurrent radiation therapy. Restaging PET scan showed persistent metastatic disease. She was switched to Cisplatin/Taxol after having hypersensitivity reaction to Carboplatin. She received 5 cycles with progression of disease in the follow up scans. She then received Nivolumab for 2 cycles. The patient then opted for comfort directed care given worsening functional status and progression of disease on repeat imaging. Secondary cardiac tumors are very rare and not extensively studied in oncology. Therefore, optimal management is not entirely clear. It is extremely rare for vulvar cancer to metastasize to the heart and only two cases have been reported in the literature. However, vulvar cancer metastasizing to the right ventricular cavity and endocardium has not been described before. We believe that this is the first ever such reported case.

Casual or Causal? Two Unique Cases of Hodgkin’s Lymphoma: A Case Report and Literature Review

Case series Patient: Male, 38 • Male, 30 Final Diagnosis: Hodgkin’s lymphoma Symptoms: Lymphadenopathy • shortness of breath Medication: — Clinical Procedure: — Specialty: Oncology Objective: Rare disease Background: Immunosuppressive diseases and therapies have long been connected to risk of malignancies, especially lymphoma. With some diseases and drugs, the association is well established but the data is mostly anecdotal because of the rarity of the situation. Case Reports: We present 2 rare cases. The first patient had psoriasis, was on etanercept, and developed Hodgkin’s lymphoma. This case is rare because psoriasis and etanercept do not usually cause lymphoma, and if they do, it is predominantly Epstein-Barr virus-positive non-Hodgkin’s lymphoma. The second patient had acquired immune deficiency syndrome (AIDS) and developed Hodgkin’s lymphoma while on highly active antiretroviral therapy (HAART). This case is rare because AIDS mostly causes Kaposi’s sarcoma or non-Hodgkin’s lymphoma due to immunosuppression, but whether it is AIDS or HAART therapy that leads to development of Hodgkin’s lymphoma in these patients is not clear. Conclusions: Immunosuppression seems to be the primary culprit leading to lymphomas in these cases. The exact mechanism is still not completely understood.

Analysis of Efficacy and Tolerability of Bruton Tyrosine Kinase Inhibitor Ibrutinib in Various B-cell Malignancies in the General Community: A Single-center Experience

Background Ibrutinib, an irreversible inhibitor of Bruton tyrosine kinase (BTK), is a novel drug that has shown significant efficacy and survival benefit for treatment of various B‐cell malignancies. The primary objective of the present study was to investigate the efficacy of ibrutinib therapy in various B‐cell malignancies in the general community. The secondary objectives included studying the adverse effects, ibrutinib‐induced peripheral lymphocytosis, and effect on immunoglobulin levels. Patients and Methods The present study was a retrospective observational cohort analysis conducted at Abington Jefferson Health. The clinical response was determined from the hematologists assessment and evaluated independently using the response criteria for each B‐cell malignancy. Adverse effects were graded according to the Common Terminology Criteria for Adverse Events, version 4.0. The Wilcoxon signed‐rank test was used to compare immunoglobulin levels before and after ibrutinib. Forty five patients with B‐cell malignancies and receiving ibrutinib therapy were eligible. Results The median age was 73 years (range, 49‐96 years), and 84.4% of the patients had received ≥ 1 previous therapy. The best overall response rate of all cohorts combined was 63.8%. The greatest overall response rate was observed in patients with chronic lymphocytic leukemia or small lymphocytic lymphoma (76.1%), followed by those with Waldenström macroglobulinemia (75%). Of the 45 patients, 88.9% experienced adverse effects. Antiplatelet activity of ibrutinib was most commonly observed (30.5%). Of note, 5 patients (11%) developed new‐onset atrial fibrillation after drug initiation. Peripheral lymphocytosis after drug initiation was observed in most patients, with a peak level at 1 month (median lymphocyte count, 2.7 × 103 cells/&mgr;L). Although the IgG levels at 3, 6, and 12 months had decreased (P = .01 for all) compared with the levels before ibrutinib, the IgA levels had not increased at 3, 6, 12, and 24 months (P = .6, P = .5, P = .3, and P = .9, respectively). Conclusion Ibrutinib is a highly effective and tolerable drug for B‐cell malignancies in the general community. In contrast to the previously reported rate of 5% to 7%, we observed a higher rate (11%) of atrial fibrillation, which might have resulted from the smaller sample size in the present study and the multiple comorbidities. Nonetheless, this treatment‐limiting side effect requires further elucidation. Paradoxical lymphocytosis at the outset of therapy was a common and benign finding. In conjunction with the reported trials, the IgG levels decreased in the first year of continued therapy. However, the IgA levels did not increase, even after 2 years of therapy. Micro‐Abstract Ibrutinib, a novel Bruton tyrosine kinase inhibitor, has revolutionized the treatment of various B‐cell malignancies. In this retrospective study, we analyzed the data of 45 patients with various B‐cell malignancies to determine ibrutinibs clinical efficacy and adverse effects in a real‐world setting. Results showed an excellent efficacy and a favorable toxicity but a higher incidence of atrial fibrillation.

Continuous diphenhydramine infusion and imatinib for KIT-D816V-negative mast cell activation syndrome: a case report

BackgroundWe present the first full case report of the treatment of mast cell activation syndrome with continuous diphenhydramine infusion, which resulted in the improvement of anaphylactic reactions and a decrease in hospital readmission. Furthermore, the patient received imatinib in the absence of the KIT-D816V mutation, which led to further improvement of quality of life. Currently, we are trying to wean this patient off diphenhydramine; if successful, this attempt will represent the first reported case.Case presentationAn 18-year-old white girl presented with a flare of mast cell activation syndrome and received epinephrine and steroids. She had failed multiple previous therapies, and her quality of life was affected due to two to three flares/week. She was started on continuous diphenhydramine infusion and imatinib, which led to a decrease in hospital admissions and marked improvement in her quality of life.ConclusionsContinuous diphenhydramine infusion can provide promising outcomes following the failure of intermittent antihistamine dosing in patients with severe mast cell activation syndrome. Initiating continuous diphenhydramine infusion may be helpful in an intensive care setting when the patient is particularly prone to anaphylaxis and/or the resources needed to manage anaphylaxis are not available outside the intensive care unit. Furthermore, imatinib provides benefits in KIT-D816V-negative mast cell disorders due to other unknown mutations.

Case series of multiple primary cancers in single individuals: diagnostic and therapeutic dilemmas

ABSTRACT Background and Objectives: Cancer recurrence represents treatment failure; the development of new primary tumors is suggestive of persistent exposure to etiological risk factors or genetic predisposition due to mutations in multiple cell lines. Case presentation/Design/Methods: The first case is a 65-years-old Caucasian male who presented with esophageal and lung cancer diagnosed synchronously. Smoking was the common risk factor for both cancers, underscoring field cancerization. The diagnosis and management was a challenge and different from either cancer presenting alone. Multidisciplinary approach was used and led to good outcomes. The second case is a 72-years-old Caucasian male presenting a rare dilemma of genetic mutation leading to multiple primary gastrointestinal cancers in a single individual. The gene explaining this group of cancers has not been diagnosed yet and the field needs to be explored further. Conclusion: Multiple primary cancers can be secondary to a common environmental risk factor or genetic mutations.

An extensive unprovoked left lower extremity deep vein thrombosis secondary to an anatomical anomaly: a case of May-Thurner syndrome

May-Thurner syndrome (MTS) also known as Cockett’s syndrome is a rare condition responsible for 2%-3% of all cases of deep venous thrombosis (DVT). The thrombosis results from mechanical compression of the left common iliac vein against the body of the fifth lumbar vertebra by the right common iliac artery. Repetitive hyperplasia of the venous wall by compression results in spur formation that in turn causes venous flow obstruction and results in the DVT. Our case is a young female who had acute extensive proximal DVT due to MTS that was successfully managed using mechanical thrombectomy with a venous stent. MTS although a rare entity should be suspected especially in young patients with unilateral DVT with extensive clots especially on left lower extremity without any antecedent risk factors.

Severe Refractory Hypocalcemia in a Patient with Metastatic Prostate Carcinoma Following Denosumab Injection

An 86-year-old male with a history of metastatic castrate sensitive prostate cancer received a single dose of Denosumab for his bony metastases. Two weeks later, he presented to the hospital due to left foot cellulitis and was incidentally found to have profound hypocalcemia whereas his serum calcium was normal at the time of Denosumab injection. A thorough workup was undertaken which showed severe Vitamin D deficiency. He was diagnosed with Denosumab induced hypocalcemia with underlying Vitamin D deficiency which was refractory to supplemental calcium and Vitamin D. This case demonstrates the potential of Denosumab to cause profound hypocalcemia which can be resistant to therapy. Bone metastasis is a common clinical encounter and Denosumab is an effective therapy to prevent skeletal related events (SRE). Therefore, given its widespread use, it is extremely important to identify and treat risk factors that may aggravate hypocalcemia when treated with Denosumab.

Eculizumab refractory thrombotic thrombocytopenic purpura secondary to post-endoscopic retrograde cholangiopancreatography pancreatitis in a patient

Thrombotic thrombocytopenic purpura (TTP) is a rare multisystem microvascular disorder, which is characterized by pentad of thrombocytopenia, microangiopathic hemolytic anemia, and organ dysfunction due to occlusive thrombi. The proposed pathophysiology involves an imbalance between unusually large von Willebrand factor multimers and the cleaving protease ADAMTS13. Acute pancreatitis is a well-described consequence of TTP, but TTP secondary to acute pancreatitis is a rare phenomenon. We present a patient who developed TTP due to post-ERCP pancreatitis with hematologic, cardiovascular, pulmonary, and renal complications and is the first case of this kind. Despite early initiation of therapy, the patient did not recover making it among the 10% of cases of TTP that prove fatal despite appropriate therapy.