This article explores the rare case of an 82-year-old man diagnosed concurrently with essential thrombocythemia and smoldering multiple myeloma (SMM). The limited existing literature on individuals harboring both myeloproliferative neoplasm (MPN) and monoclonal gammopathy (MG) is of significant interest due to the distinct origins of these malignancies. The etiology of MG in MPN patients remains elusive, leading to speculation about a potential relationship or interplay between the two conditions. This unique case prompts a deeper exploration of the mechanisms underlying the coexistence of JAK2-positive MPN and SMM. It underscores the importance of tailored therapeutic strategies that carefully consider the inherent risks and potential adverse outcomes associated with these specific malignancies, thereby warranting further clinical research.

OBJECTIVE: Multiple myeloma (MM) is a plasma cell dyscrasia leading to proliferation of monoclonal plasma cells. Ocular involvement in multiple myeloma is uncommon but can occur. The ocular manifestations of MM may include the cornea, uvea, and retinal vasculature. We present a rare case of autoimmune retinopathy associated with smoldering MM.
METHODS: A 76-year-old female with no significant past medical or ocular history presented with four months of worsening vision, difficulty with night driving, and loss of peripheral vision. Examination was notable for pallor of the optic nerves and vascular attenuation. Visual field testing demonstrated significant and progressive field loss in both eyes. An electroretinogram was extinguished under all conditions. Serum protein electrophoresis showed a significant elevation of IgG with an M-spike, and a subsequent bone marrow biopsy was performed showing 12.5% plasma cells, consistent with the diagnosis of MM. CAR antibody testing was positive for anti-enolase, anti-GAPDH, and anti-Rab6 antibodies, consistent with autoimmune retinopathy.
CONCLUSIONS: Autoimmune retinopathy associated with MM is exceedingly rare. Management of this condition is challenging, as treatment of the underlying disease does not often lead to improvement in visual symptoms. Ultimately, visual prognosis is very poor, and both patients and clinicians should be aware of the guarded visual potential.
CONCLUSIONS: The association of autoimmune retinopathy with multiple myeloma is rare. It is crucial for physicians to be aware of such manifestations to ensure timely and appropriate diagnosis and management for patients.

Amyloidosis is a leading cause of infiltrative cardiomyopathy and in turn heart failure with preserved ejection fraction. Amyloidosis is mainly classified into amyloid light chain (AL) or primary amyloidosis and transthyretin amyloidosis (ATTR) that is subdivided into wild-type ATTR (ATTRwt) and hereditary or familial transthyretin-related amyloidosis (hATTR). Moreover, strain preservation pattern in the left ventricular apex in echocardiography suggests cardiac amyloidosis and cardiac magnetic resonance (CMR) could identify an infiltrative process. Similarly, the radiotracer uptake of technetium-99m pyrophosphate by myocardium could indicate transthyretin accumulation. In contrast, serum-free light chain (FLC) alongside serum and urine immunoelectrophoresis could indicate AL amyloidosis. Here, we present a case of a 60-year-old male with a classical apical sparing on echocardiography but with an unremarkable CMR and technetium-99 m pyrophosphate.

To date, there have been nine reported instances of coinciding smoldering multiple myeloma (SMM) and primary biliary cholangitis (PBC). The term SMM was coined in 1980 to describe low-severity multiple myeloma cases, a hematologic neoplasia that involves the malignant proliferation of plasma cells. PBC is an autoimmune disorder targeting the intrahepatic bile ducts and is characterized by elevated anti-mitochondrial antibodies and often resulting in autoimmune liver cirrhosis. Currently, there is no plausible rationale for the coincidence of SMM and PBC in patients. This report investigates the relationship between SMM and PBC in a Hispanic 49-year-old female residing in the United States and attempts to determine the possible genetic and biochemical causes of this coincidence.

This case illustrates clinical, histopathological, immunohistochemical, and molecular pathological diagnostic testing of smoldering myeloma with atypical ophthalmic manifestations. In our case, the choroidal lesion presented as a solitary manifestation of a systemic disease. Choroidal lesions of monoclonal plasma cells are extremely rare and should be included in the differential diagnosis of amelanotic choroidal lesions, even if the histopathological examination of the primary lesion is not informative. Clinical course, immunohistochemistry, and molecular pathology are essential components of the diagnostic pathway.
Dieser Fall schildert klinische, histopathologische, immunhistochemische und molekulare pathologische Diagnoseverfahren beim Smoldering Myelom mit einem atypischen ophthalmologischen Erscheinungsbild. In unserem Fall war die Aderhautläsion die einzige Manifestation einer systemischen Erkrankung. Aderhautläsionen monoklonaler Plasmazellen sind extrem selten und sollten bei der Differenzialdiagnose amelanotischer Aderhautläsionen berücksichtigt werden, selbst wenn eine histopathologische Untersuchung der primären Läsion keinen Aufschluss liefert. Der klinische Verlauf, die Immunhistochemie sowie die molekulare Pathologie sind essenzielle Komponenten des diagnostischen Verlaufs.

BACKGROUND: Smoldering multiple myeloma (SMM) is an asymptomatic plasma cell proliferative disorder that can progress to multiple myeloma (MM). Amyloidosis (light chain) (AL) is the most common form of systemic amyloidosis. There are few reports of SMM coexisting with AL involving the digestive tract.
METHODS: A 63-year-old woman presented with lower limb edema, abdominal distension, abdominal pain, and hematochezia. Gastroscopy showed gastric retention, gastric angler mucosal coarseness, hyperemia, and mild oozing of blood. Colonoscopy showed hyperemic and edematous mucosa of the distal ascending colon and sigmoid colon with the presence of multiple round and irregular ulcers, submucosal ecchymosis, and hematoma. Gastric and colonic tissue biopsy confirmed the diagnosis of AL by positive Congo red staining. MM was confirmed by bone marrow biopsy and immunohistochemistry. The patient had no hypercalcemia, renal dysfunction, anemia, bone lesions or biomarkers of malignancy defined as plasma cells > 60% in bone marrow. Additionally, no elevated serum free light chain ratio, or presence of bone marrow lesions by magnetic resonance imaging (SLiM criteria) were detected. The patient was finally diagnosed with SMM coexisting with AL. She received chemotherapy and was discharged when the symptoms were relieved. She is doing well at nearly five years of follow up.
CONCLUSIONS: This case highlights that high index of suspicion is required to diagnose gastrointestinal AL. It should be suspected in elderly patients with endoscopic findings of granular-appearing mucosa, ecchymosis, and submucosal hematoma. Timely diagnosis and appropriate therapy can help to improve the prognosis of these patients.

While several case reports suggest an association between sarcoidosis and multiple myeloma (MM), few cases involve smoldering MM. We report a case of sarcoidosis and smoldering MM discovered simultaneously in a patient admitted for hypercalcemia. Initial tests raised suspicion for sarcoidosis and MM, prompting invasive testing. Surgical lung biopsy revealed necrotizing granulomas, which could represent sarcoidosis in the appropriate setting. Thus, sarcoidosis was diagnosed following a negative infectious workup. Bone marrow biopsy revealed 13% plasma cells leading to subsequent diagnosis of smoldering MM. This case demonstrates the challenge of determining disease activity when other causes of CRAB symptoms are present.

We have reported an extremely rare case of a frontal convexity tumor diagnosed as IgG4-related disease (IgG4-RD) with unique neuroradiological images.
A 64-year-old man with a history of monoclonal gammopathy of undetermined significance and conservative treatment had presented with a left facial spasm. Computed tomography showed a high-density round tumor with perifocal edema in the right frontal convexity. Magnetic resonance imaging demonstrated unique findings, including low signal intensity on T1- and T2-weighted, fluid-attenuated inversion recovery, and diffusion-weighted images, with slight gadolinium enhancement. The tumor was totally removed via right frontal craniotomy. It had been located in the subdural space, was not adherent to the dura, and was less vascular than meningiomas. Histological investigation demonstrated plasma cells that were strongly positive for IgG4 and contained κ and λ light chains at a ratio of 1.5:1. The serum IgG4 level was elevated. The tumor met the diagnostic criteria for IgG4-RD. The patient was followed up for 3 years during postoperative adjuvant steroid therapy. The steroid therapy was discontinued, and during the next 4 years, neither tumor recurrence nor symptoms were observed.
Intracranial IgG4-RD with smoldering monoclonal gammopathy of undetermined significance is extremely rare. We reviewed the differential diagnosis of plasma cell granuloma and plasmacytoma, therapeutic implications, and clinical outcomes. Complete resection of a conspicuous and solitary IgG4-RD lesion in the frontal convexity is simple and could provide a cure with less-aggressive adjuvant therapy.

Acquired von Willebrand syndrome is a rare bleeding disorder often secondary to an underlying lymphoproliferative disorder. We report a case in whom response of both the acquired von Willebrand syndrome and smoldering multiple myeloma persist 14 months after daratumumab treatment discontinuation.

OBJECTIVE: A case of interference of monoclonal protein (M-protein) on thrombin time (TT) test in a 39-year-old Caucasian male patient is presented.
METHODS: Coagulation screening tests were performed where altered results only for TT result (>150 seconds) and activated partial thromboplastin time (aPTT) result (36 seconds) were measured. Further specific coagulation testing included measurement of individual coagulation factors FII, FV, FVII, FVIII, FIX, FX, FXI, and FXII. Diagnostic steps in detection and identification of monoclonal protein included serum protein electrophoresis and immunofixation (both serum and urine specimen).
RESULTS: Monoclonal protein immunoglobulin G kappa detection and identification in serum and urine clarified the situation.
CONCLUSIONS: Unexpectedly altered results of screening coagulation tests without any appropriate clinical signs and symptoms in a patient without any anticoagulant therapy needs to be critically considered in the context of extended next diagnostic steps in order to clarify the cause of pathological test results.