Hyperammonemic Encephalopathy: A Rare Presentation of Relapsed Multiple Myeloma.

Hyperammonemia is a rare cause of encephalopathy in multiple myeloma in the absence of hepatic involvement. This is the only reported case of a 74-year-old man who presented with multiple myeloma and achieved complete remission but developed hyperammonemia afterward. He was aggressively treated with a combination of chemotherapy and immunotherapy, with a resolution of his encephalopathy; however, within one month, he relapsed with encephalopathy. He ultimately decided to pursue comfort-care measures. The authors conclude that hyperammonemia in multiple myeloma is a rare but important differential in patients with encephalopathy of unknown causes. Aggressive treatment is of the utmost importance due to the high mortality associated with the condition.

Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature.

Hereditary platelet delta (δ)-storage pool deficiency is a rare condition in which there are fewer dense granules in platelets disrupting primary hemostasis. It can cause a mild-moderate bleeding tendency with normal coagulation studies; hence, it is an underdiagnosed diagnostic challenge. The authors present three patients with hereditary platelet delta (δ)-storage pool deficiency who had heavy menstrual bleeding, excessive bleeding following surgery, mucocutaneous bleeding, and a bleeding score greater than or equal to 6. These cases reveal the susceptibility of underdiagnosing platelet disorders and the significance of utilizing a bleeding assessment tool to help guide further workup with transmission electron microscopy to visualize the fewer dense granules in platelets. Although bleeding is typically moderate, it can be severe in certain scenarios, like after mucosal surgeries, and can lead to death, highlighting the importance of the condition's recognition and prophylactic treatment.

Phenazopyridine-Induced Methemoglobinemia in a Jehovah's Witness Treated with High-Dose Ascorbic Acid Due to Methylene Blue Contradictions: A Case Report and Review of the Literature.

Methemoglobinemia is an acute medical emergency that requires prompt correction. Physicians should have a high degree of suspicion of methemoglobinemia in cases that present with hypoxemia that does not resolve with supplemental oxygenation, and they should confirm this suspicion with a positive methemoglobin concentration on arterial blood gas. There are multiple medications that can induce methemoglobinemia, such as local anesthetics, antimalarials, and dapsone. Phenazopyridine is an azo dye used over-the-counter as a urinary analgesic for women with urinary tract infections, and it has also been implicated in causing methemoglobinemia. The preferred treatment of methemoglobinemia is methylene blue, but its use is contraindicated for patients with glucose-6-phosphatase deficiency or those who take serotonergic drugs. Alternative treatments include high-dose ascorbic acid, exchange transfusion therapy, and hyperbaric oxygenation. The authors report a case of a 39-year-old female who took phenazopyridine for 2 weeks to treat dysuria from a urinary tract infection and subsequently developed methemoglobinemia. The patient had contraindications for the use of methylene blue and was therefore treated with high-dose ascorbic acid. The authors hope that this interesting case promotes further research into the utilization of high-dose ascorbic acid for managing methemoglobinemia in patients who are unable to receive methylene blue.

De-Novo B-Cell Prolymphocytic Leukemia.

B-cell prolymphocytic leukemia (B-PLL) is a rare B-cell neoplasm that typically presents with splenomegaly, a rising white blood cell count, and may or may not have B symptoms. The diagnosis usually requires a bone marrow biopsy and aspirate with flow cytometry and cytogenetic studies. At least 55% of the lymphocytes in the peripheral blood must be prolymphocytes to be defined as B-PLL. A thorough differential diagnosis would include mantle cell lymphoma, chronic lymphocytic leukemia (CLL) with prolymphocytes, hairy cell leukemia, and splenic marginal zone lymphoma. B-PLL is managed with regimens utilized for CLL, such as ibrutinib and rituximab but is tailored for each individual. The authors report a rare case of B-PLL in a patient with no known history of CLL. The authors discuss this entity in context of the 2017 and 2022 World Health Organization (WHO) classifications, the latter of which no longer recognizes B-PLL as a distinct entity. The authors hope that this article helps practitioners with the diagnosis and treatment of B-PLL. Perhaps with better recognition, and better documentation of histopathologic features of these rare cases going forward, it may prove to be a distinct entity again in future classifications.

Testosterone Usage Leading to Pulmonary Embolisms and Deep Vein Thrombosis: A Case Report and Review of the Literature.

Androgen usage has widely increased in recent times via prescribed and unprescribed means. Testosterone is a popular androgen taken by both athletes and the general population. While there is some evidence of androgens being thrombogenic, we report on a 19-year-old male who presented to the hospital after the usage of testosterone for one month, leading to the development of multiple pulmonary emboli and deep vein thrombosis. The authors hope to elucidate the relationship between testosterone usage and thrombosis formation.

Acquired Factor X Deficiency without Amyloidosis Presenting with Massive Hematuria: A Case Report and Review of the Literature.

Acquired factor X deficiency is a rare diagnosis, especially without the association of other co-existing conditions such as amyloidosis. The authors report the case of a 34-year-old male with severe frank hematuria found to have markedly prolonged prothrombin time and activated partial thromboplastin time. A mixing study showed correction utilizing normal plasma and a coagulation panel testing revealed decreased factor X activity. The patient was treated with multiple blood transfusions, fresh frozen plasma, high-dose pulse steroids, and rituximab. The patient's condition improved during his 21-day hospital stay and was followed up every 2 weeks for 3 months. The patient's factor X level recovered after two weeks of discharge with no other hemorrhagic episodes.

Pathological Complete Response of Metastatic Clear Cell Renal Carcinoma with Pembrolizumab and Axitinib: A Case Report and Review of Literature.

The role of cytoreductive nephrectomy has become unclear since the introduction of immunotherapy which is now the backbone of the treatment for metastatic renal cell carcinoma. Different combinations are used based on the prognosis. Achieving a complete response would be ideal and includes radiographic disappearance of lesions. However, there have been a few reported cases of pathological complete response with persistent radiographic evidence of cancer. The authors report a case of pathological complete response despite persistent radiographic evidence of residual disease in a patient with metastatic renal cell carcinoma treated with pembrolizumab and axitinib. The patient subsequently underwent cytoreductive nephrectomy after the 13th dose of pembrolizumab. The resected mass consisted of scar tissue with no viable tumor cells seen on pathology but only scar tissue. This case reveals that persistent radiographic evidence of the tumor may be explained by scar tissue, challenging the role of cytoreductive nephrectomy in the era of immunotherapy.

Stage 4 Cytokine Release Syndrome Caused by the First Dose of Nivolumab and Ipilimumab Combination Therapy in a Patient with Metastatic Melanoma Successfully Treated with Methylprednisolone, Tocilizumab, and Etanercept.

The authors report the first case of stage 4 cytokine release syndrome (CRS) (graded by the National Cancer Institute Common Terminology Criteria for Adverse Effects scale) involving a patient with advanced metastatic melanoma who was treated with the combination of two monoclonal antibodies, nivolumab (anti-programmed cell death receptor 1 inhibitor [PD-1]) and ipilimumab (a cytotoxic T lymphocyte-associated antigen 4 inhibitor [CTLA-4]) after her first dose of both. The patient was treated initially with methylprednisolone and tocilizumab but was refractory to treatment. A trial of etanercept was initiated due to her elevated levels of TNF-α which elicited a satisfactory response. Monoclonal antibody therapy is a new tool for the treatment of many cancers, and therefore there may be a subsequent rise in the cases of CRS and this case exemplifies a treatment algorithm. Utilizing levels of cytokines assists in tailoring treatment such as in this case where etanercept, a TNF-α inhibitor, was utilized due to the patient's elevated levels of TNF-α.

Acute Hyperhemolysis Syndrome in a Patient with Known Sickle Cell Anemia Refractory to Steroids and IVIG Treated with Tocilizumab and Erythropoietin: A Case Report and Review of Literature.

Patients with sickle cell anemia often receive multiple red blood cell (RBC) transfusions during their lifetime. Hyperhemolysis is a life-threatening phenomenon of accelerated hemolysis and worsening anemia that occurs when both transfused RBCs and autologous RBCs are destroyed. The level of hemoglobin post-transfusion is lower than pre-transfusion levels, and patients are usually hemodynamically unstable. Hyperhemolysis must be differentiated from a delayed hemolytic transfusion reaction during which destruction of transfused RBC is the cause of anemia. Hyperhemolysis syndrome can be differentiated into acute (within seven days) and chronic forms (after seven days) post-transfusion. The authors present a case of acute hyperhemolysis syndrome in a patient with sickle cell anemia refractory to steroids and IVIG, which are the treatment of choice. The patient was treated with tocilizumab, combined with supportive measures of erythropoietin, iron, vitamin B12, and folate.

Spontaneous Tumor Lysis Syndrome in a Patient with Bulky Chronic Lymphocytic Leukemia Diagnosed after Resolution of Symptoms.

Tumor lysis syndrome (TLS) is an oncologic emergency characterized by the destruction of tumor cells leading to an influx of large amounts of uric acid, potassium, and phosphorus into systemic circulation. It most often occurs after the initiation of cytotoxic therapy in high-grade lymphomas and leukemias; however, rarely it may occur spontaneously. The authors report a case of spontaneous tumor lysis causing electrolyte abnormalities and acute kidney injury in a patient with subsequently diagnosed large chronic lymphocytic leukemia tumor burden. Spontaneous TLS can be the first presentation of underlying malignancy; therefore, physicians should be aware of the associated findings.

The Development of Immune Thrombocytopenia Due to COVID-19 Presenting as Menorrhagia.

Immune thrombocytopenia (ITP), also known as immune thrombocytopenic purpura, is a hematological disorder characterized by a decreased platelet count, predisposing patients to bleeding. Coronavirus disease 2019 (COVID-19) has been linked to multiple cases of newly diagnosed ITP and is usually found in moderate-to-severe infections, peaking in children and elderly adults. Menorrhagia is the medical term for menstrual periods with abnormally heavy or prolonged bleeding occurring at regular intervals or prolonged uterine bleeding lasting more than seven days. Here, we report the case of a 23-year-old African American female who presented with the chief complaint of menorrhagia and was subsequently diagnosed as having ITP induced by an asymptomatic COVID-19 infection.

Cyclical Thrombocytopenia Synchronized With the Patient's Menstrual Cycle Treated With Danazol.

Cyclical thrombocytopenia (CTP) is a very rare condition and often misdiagnosed as immune thrombocytopenia (ITP) due to similar features existing between the two. When evaluating a patient for the possible diagnosis of ITP, CTP must be high on the differential diagnosis. The main difference between the two conditions is that CTP is usually unresponsive to the treatment given to ITP and will ultimately display a cyclical nature with periods of low, normal and elevated platelets. As of date, there are only 70 cases in the literature. However, this number may be misrepresented due to the difficulty in diagnosis. The authors report a case of a 36-year-old woman who was misdiagnosed with ITP and underwent unnecessary treatment with corticosteroids, rituximab, intravenous immunoglobulins, and a splenectomy. A diagnosis of CTP was made after extensive review and the authors aim to bring awareness of this uncommon condition.

Diabetic Ketoacidosis as the Initial Presenting Symptom of Pancreatic Adenocarcinoma: A Discussion about Screening Utilizing ENDPAC Scoring Coupled with CT Scans and Endoscopic Ultrasound.

Pancreatic adenocarcinoma is often discovered at an advanced stage due to a lack of early symptomology, resulting in this being the fourth leading cause of cancer-related death in the USA. The relationship between diabetes mellitus and pancreatic cancer has been known for many years; however, it is not well understood. Studies have suggested that long-standing type 2 diabetes mellitus can increase the risk of pancreatic cancer by 1.5-2.0-fold. However, patients with new-onset diabetes over 50 years of age have an 8-fold higher risk of pancreatic cancer. Evidence has shown that pancreatic cancer causes diabetes, with the majority being new onset. Diabetic ketoacidosis, which occurs in long-term hyperglycemia, as an initial presentation of pancreatic adenocarcinoma is rare, and only a few cases have been reported. It is postulated that pancreatic cancer prevents insulin-producing cells of the pancreas from responding to insulin resistance. The enriching new-onset diabetes for pancreatic cancer (ENDPAC) score may be utilized as a screening tool for pancreatic carcinomas as an early diagnosis may lead to cure by surgery instead of the grave prognosis associated with it. In this case report, we discuss a 52-year-old female presenting with symptoms of diabetic ketoacidosis who was then subsequently found to have stage-4 pancreatic adenocarcinoma. We concluded that if a patient presents with new-onset diabetes, abdominal imaging with CT scans and endoscopic ultrasound may be warranted to rule out pancreatic carcinoma.

Disseminated Intravascular Coagulation Due to Drinking Tonic Water.

Quinine has been used worldwide to treat malaria; however, it is now used as an agent for night-time muscle cramping. The compound, derived from Cinchona tree bark, is found in antimalaria medication, supplements for leg cramping, and beverages such as tonic water and bitter lemon. Quinine, however, is not without its side effect profile which includes a wide range of ailments ranging from nausea to disseminated intravascular coagulation. The authors present a case of a 35-year-old man diagnosed with disseminated intravascular coagulation due to an excessive intake of tonic water because his friend told him that it would help alleviate nighttime leg cramping. We strive to inform physicians about the side effect profile of quinine and stress that a pertinent history must be elicited in patients with unknown causes of disseminated intravascular coagulation.

Hyperleukocytosis and Pseudohyperkalemia in a Patient With Severe SARS-COVID 19 Unmasks Chronic Lymphocytic Leukemia.

Coronavirus disease 2019 (COVID-19) has caused significant morbidity and mortality in a vast majority of the patient population, especially those with malignancies. Chronic lymphocytic leukemia (CLL) is the most common leukemia in adults and is often an indolent disease. High white blood cell counts greater than 120 k/cumm in chronic lymphocytic leukemia may be implicated in cases of COVID-19. Hyperleukocytosis leads to falsely elevated potassium levels due to cell fragility. Pseudohyperkalemia occurs when elevated potassium is present due to potassium movement out of cells during or after a blood sample is drawn. Pseudohyperkalemia may be suspected when elevated potassium is present in asymptomatic patients with no corresponding electrocardiogram findings. The authors present a case of hyperleukocytosis and pseudohyperkalemia in a patient whose COVID-19 infection unmasked CLL.