A Hypercalcemic Enigma: A Rare Case of Bone Marrow Sarcoidosis.

Sarcoidosis is a multisystem inflammatory disease involving granuloma formation. The exact etiology of the disease remains unknown. While the lungs are the most commonly involved organs in sarcoidosis, bone marrow-only involvement is quite rare. As bone marrow-only involvement can have a presentation that closely resembles multiple myeloma, a significant diagnostic dilemma can occur as the treatment for sarcoidosis is different compared to multiple myeloma. We present a case of a 62-year-old female who presented with worsening lower extremity weakness and fatigue. She was found to have new-onset hypercalcemia, normocytic anemia, and lytic bony lesions. Extensive laboratory workup for multiple myeloma was negative with bone marrow biopsy showing non-caseating granulomas, thus diagnosing the patient with sarcoidosis involving the bone marrow.

The Role of Bispecific Antibodies in Relapsed Refractory Multiple Myeloma: A Systematic Review.

Multiple myeloma is a heterogeneous clonal malignant plasma cell disorder, which remains incurable despite the therapeutic armamentarium's evolution. Bispecific antibodies (BsAbs) can bind simultaneously to the CD3 T-cell receptor and tumor antigen of myeloma cells, causing cell lysis. This systematic review of phase I/II/III clinical trials aimed to analyze the efficacy and safety of BsAbs in relapsed refractory multiple myeloma (RRMM). A thorough literature search was performed using PubMed, Cochrane Library, EMBASE, and major conference abstracts. A total of 18 phase I/II/III studies, including 1283 patients, met the inclusion criteria. Among the B-cell maturation antigen (BCMA)-targeting agents across 13 studies, the overall response rate (ORR) ranged between 25% and 100%, with complete response/stringent complete response (CR/sCR) between 7 and 38%, very good partial response (VGPR) between 5 and 92%, and partial response (PR) between 5 and 14%. Among the non-BCMA-targeting agents across five studies, the ORR ranged between 60 and 100%, with CR/sCR seen in 19-63%, and VGPR in 21-65%. The common adverse events were cytokine release syndrome (17-82%), anemia (5-52%), neutropenia (12-75%), and thrombocytopenia (14-42%). BsAbs have shown promising efficacy against RRMM cohorts with a good safety profile. Upcoming phase II/III trials are much awaited, along with the study of other agents in concert with BsAbs to gauge response.

Copper deficiency, a rare but correctable cause of pancytopenia: a review of literature.

INTRODUCTION: Copper is increasingly being recognized as a vital mineral required by both animals and humans. It plays a vital role in many metabolic processes such as cellular respiration, iron oxidation, and hemoglobin synthesis. Copper deficiency, which can be hereditary or acquired, can lead to a wide spectrum of disease processes such as ringed sideroblastic anemia, myelodysplasia, and pancytopenia. Timely identification and management of copper deficiency is necessary to prevent irreversible complications. AREAS COVERED: Our study focuses on prevalence, etiology, pathophysiology, complications, and treatment of copper deficiency. EXPERT OPINION: Copper deficiency is frequently underrecognized as the cause of anemia, neutropenia, and bone marrow dysplasia. As it is potentially treatable, it should always be kept in the differentials when patients present with neurological and hematological abnormalities.

Changing incidence and survival of desmoplastic small round cell tumor in the USA.

The incidence and prognosis of desmoplastic small round cell tumor (DSRCT) is inadequately understood. Survival analysis for DSRCT has not been investigated in a population-based study. We conducted a retrospective cohort study using the Surveillance, Epidemiology, and End Results (SEER) 9 Registry (1975-2018). Annual percent changes in incidence were estimated using SEER*Stat, and risk ratios were estimated using Poisson regression. Cox regression models were constructed to estimate the hazard ratio for survival at 5 years. The incidence rate of DSRCT has been rising in the last two decades. Men had a higher age-adjusted incidence rate, and nonmetropolitan counties had a higher incidence rate than metropolitan counties. Blacks had a higher risk of being diagnosed with DSRCT than whites. The observed survival at 12, 36, and 60 months was 81%, 39.9%, and 23.4%, respectively. Those >70 years had a poorer survival than those <60 years (P < 0.001). Compared to surgery with chemotherapy, surgery with chemoradiotherapy was linked to a 53% lower risk of mortality (P < 0.001). We conclude that the DSRCT incidence has been increasing since 2000 with a white male predominance. Gender doesn't affect survival in DSRCT, and surgery combined with chemoradiotherapy improves survival compared to surgical management with chemotherapy alone.

Posttransplant lymphoproliferative disorder in a kidney transplant recipient: a case of Epstein-Barr virus-positive primary central nervous system lymphoma.

Posttransplant lymphoproliferative disorders (PTLDs) are potentially life-threatening complications of chronic immunosuppression in patients who receive solid organ transplants or allogeneic hematopoietic cell transplantation. Most PTLD cases are associated with Epstein-Barr virus (EBV) serology, and their incidence is typically higher in the first year of transplantation. Isolated EBV-positive diffuse large-cell B-cell lymphoma in the renal transplant setting has rarely been reported. Isolated EBV-positive primary central nervous system lymphoma (PCNSL) is rare, even in renal transplant patients with chronic immunosuppression. We report a case of frontal lobe EBV-positive PCNSL in a renal transplant patient who presented with left-sided weakness and was later treated with a consolidated chemotherapeutic regimen without concurrent radiotherapy.

Diffuse Large B-Cell Lymphoma Germinal Center B-Cell Subtype of the Thyroid.

Non-Hodgkin lymphoma is one of the most common hematological malignancies having both nodal and extranodal sites of involvement. The thyroid gland is one of the rarest primary sites. Most cases of primary thyroid lymphoma are diffuse large B-cell in nature; thus, aggressive and in extreme cases can rapidly lead to airway compromise, especially in patients who have been living with goiter for years. We present one such case of a 64-year-old female who presented with signs of airway compromise, requiring emergent airway intubation and surgical debulking. She was treated with emergent chemotherapy (DA-EPOCH-R regimen), without radiotherapy and this resulted in complete remission.

First Reported Case of Deglutition Syncope With Underlying Suppurative Parotitis.

Deglutition syncope and carotid sinus hypersensitivity are neurally mediated events, leading to potentially dangerous arrhythmias and cardiovascular events. Mostly related to underlying gastroesophageal or cardiovascular causes, sometimes, this might not be the case. We report the first-ever documented case of deglutition syncope with acute suppurative parotitis, which resolved after resolving the parotid gland's swelling.

Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a case report of a rare complication following Dengue-Chikungunya co-infection.

Pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré Syndrome (GBS) is characterized by weakness in cervicobrachial and oropharyngeal region, together with areflexia of upper limbs. Being an uncommon variant, it is often misdiagnosed as other neurological conditions resembling GBS. Although most of the cases occur as a post-infectious complication, no reports describing its development following dengue-chikungunya co-infection have been documented. A young female presented with a progressive history of swallowing difficulty, bilateral arm weakness and neck weakness. Three weeks earlier, she was presented with clinical features corresponding to dengue and was symptomatically treated. Currently, hypotonia and decreased muscle strength were observed in both upper limbs and neck. Detailed investigation revealed the presence of Immunoglobulin M (IgM) antibodies against dengue antigen (NS 1) and Chikungunya virus (CHIKV), confirming the possibility of previous dengue-chikungunya co-infection. Nerve conduction studies and electromyography of upper limbs pointed towards findings consistent with the early stages of acute motor demyelinating and possible axonal neuropathy. The detection of antiganglioside antibodies (anti-GT1a antibodies), confirmed the diagnosis of the pharyngeal-cervical-brachial variant of GBS. A five days treatment of intravenous immunoglobulin (IVIG) along with physical rehabilitation was started which led to significant improvement and the patient was discharged after 15 days. PCB is an unfamiliar variant of GBS for many clinicians. Diagnosis can be made by a thorough history, clinical examination and investigations that can rule out other potential causes of cervicobrachial and oropharyngeal weakness. It also necessitates careful monitoring and followups after mono- and co-arboviral infections to prevent any debilitating neurological complications.

Thrombotic thrombocytopenic purpura: a new menace after COVID bnt162b2 vaccine.

Thrombotic thrombocytopenic purpura (TTP) is a known menace in hematology and is quite rare in practice with known triggers. Lately, in the COVID-19 pandemic, hematology has seen a new pathology amongst which TTP associated with COVID-19 messenger RNA (mRNA) vaccine is unique. We report a case of a 69-year-old male with multiple comorbidities who presented to the hospital with severe fatigue and shortness of breath. Labs were significant for thrombocytopenia, anemia, and hemolysis with schistocytes consistent with TTP with a second dose of BNT162b2 mRNA vaccine as a likely culprit been documented.