United States

Supernumerary breast tissue is ectopic breast tissue that communicates with the breast skin, nipple or areola. Approximately 5.6% of the population exhibits supplementary breast tissue. To date, the National Comprehensive Cancer Network does not provide monitoring and management guidelines for ectopic breast DCIS. 

This report describes a woman in her 60’s with a left supernumerary nipple positioned at the inframammary fold with a recently discovered 2-cm palpable anomaly that was confirmed as an intraductal papilloma on histology. Upon surgical excision of the mass, pathology revealed a low grade ductal carcinoma in situ, estrogen receptor positive and progesterone receptor positive. The patient’s case was presented at a multidisciplinary tumor board. She was ultimately recommended for radiation therapy, followed by anti-hormone therapy.


AMA Research Challenge 2024 

Presentation of Ductal Carcinoma In Situ in a Supernumerary Nipple

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Background
POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes) is a rare condition first described in 19561. It is characterized by clonal plasma cell proliferation and peripheral neuropathy due to increased inflammatory and angiogenic mediators. Misdiagnosis as Acute or Chronic Inflammatory Demyelinating Polyneuropathy (AIDP/CIDP) often occurs due to overlapping neuropathic features. We present a rare case of POEMS syndrome initially misdiagnosed as Guillain-Barre syndrome(GBS), highlighting diagnostic challenges of this rare condition.
Case Presentation
A 44-year-old male presented with fatigue, lower extremity weakness, numbness, and tingling for 1-2 months. Previously, he experienced abdominal pain and tested positive for enteroaggregative E. coli (EAEC) diarrhea. Lumbar puncture revealed elevated protein and normal cell count in cerebrospinal fluid (CSF), leading to a presumptive diagnosis of GBS. Intravenous immunoglobulin (IVIG) was administered with mild improvement. However, after discharge, his lower extremity numbness and weakness worsened and developed numbness and weakness of the upper extremities. Additionally, he exhibited hyperpigmentation on both hands and feet and double vision without papilledema. He was admitted to the hospital with a tentative diagnosis of CIDP and was treated with intravenous solumedrol and plasmapheresis without improvement.
Further tests and imaging were conducted: Thyroid function test indicated hypothyroidism, blood tests revealed normocytic anemia, thrombocytosis, negative HIV and hepatitis B/C panels. Vascular endothelial growth factor (VEGF) was elevated to 929 pg/mL.Immunofixation detected an M spike with IgA lambda and kappa monoclonal proteins. Abdominal CT showed splenomegaly, and a skeletal survey was negative for any bone lesions. Bone marrow biopsy revealed 10-15% plasma cell myeloma with plasmablastic features and atypical megakaryocytic hyperplasia. A formal diagnosis of POEMS syndrome was confirmed. Genomic pathology shows a missense mutation of exon 17 of CSF3R at amino acid position 755, converting wild-type arginine to glutamine. 
Following the diagnosis, weekly treatment with daratumumab, cyclophosphamide, bortezomib, and dexamethasone (CyBorD) was initiated, leading to improvement. The patient’s VEGF decreased to 226 pg/mL(not normalized), thrombocytosis resolved, and lower extremity weakness improved. Despite the improvement, he remained unable to ambulate and was discharged to acute rehabilitation for further functional recovery.
Discussion
This case illustrates classic signs of POEMS syndrome initially mimicking GBS, diagnosed months after symptoms onset. POEMS syndrome should be considered in differential diagnoses for patients presenting with peripheral neuropathy and monoclonal gammopathy, especially when unresponsive to IVIG and steroids. Early recognition is crucial, as treatment for POEMS differs significantly from AIDP/CIDP, and delayed treatment can lead to progressive, debilitating neuropathy, paralysis, and ultimately, cardiorespiratory failure2.



Reference

1 Crow RS. "Peripheral neuritis in myelomatosis." Br Med J. 1956;2(5000):802-804.

2 Dispenzieri, A. (2023). POEMS syndrome. In S. V. Rajkumar & R. F. Connor (Eds.), UpToDate. 





Diagnostic Pitfalls: POEMS Syndrome's GBS Mimicry


Background: Pancreatic cancer (PC) accounts for 3% of all cancers in the United States and 7% of all cancer-related deaths. Here, we presented a metastatic PC case with an excellent response to FOLFIRINOX therapy. 
Case Presentation: In June 2014, a 62-year-old Caucasian male sought medical attention due to ongoing abdominal pain since May of the same year. An ultrasound scan followed by a CT scan of the abdomen confirmed the presence of a 4.2 cm mass in the pancreatic head, indicating obstruction in both the pancreatic and bile ducts. Multiple metastatic lesions in the liver and numerous peripancreatic lymph nodes were also detected. Biopsy specimens from both the pancreas and liver confirmed the presence of pancreatic adenocarcinoma with metastasis. He was a smoker for 20 years and smoked a packet per day. 
Due to the advanced stage of his metastatic pancreatic adenocarcinoma, surgical resection was deemed inappropriate. He commenced treatment with FOLFIRINOX (leucovorin calcium, 5-fluorouracil, irinotecan hydrochloride, and oxaliplatin) in June 2014. After three months of therapy with FOLFIRINOX, a PET (positron emission tomography) scan conducted in September 2014 revealed the size of liver metastasis reduced by around 50- 65%. In September 2015, an Endoscopic ultrasound (EUS) with fine-needle aspiration (FNA) of the pancreas came out negative for malignancy, which implies a significant positive response to FOLFIRINOX therapy.
The following six years, he had multiple follow-up scans with CT chest, abdomen, and pelvis, and they showed no disease progression.
In May 2022, a CT scan of the chest, abdomen, and pelvis revealed a notable increase in size and number of pulmonary nodules in both lungs and the hepatic lesions. Following this, he was evaluated for the ONKURE trial for better response.
Discussion: FOLFIRINOX response rate is 18.2% in metastatic pancreatic cancer patients and a complete pathologic response following FOLFIRINOX treatment is extremely rare. It has been reported in 4.5–5.9% of patients. This case report represented a remarkable response to FOLFIRINOX therapy in an advanced metastatic pancreatic cancer patient.

A Case of Excellent Response to FOLFIRINOX Therapy in a Stage IV Pancreatic Adenocarcinoma Patient

• Abstract Title 
• A Fatal case of Diffuse Large B Cell Lymphoma Presenting with Bilateral Necrotic Renal Masses. 
• Background 
• A 48-year-old woman with a history of chronic inflammatory demyelinating polyneuropathy presented to the hospital for failure to thrive. She experienced rapid decompensation during two consecutive hospitalizations; No diagnosis was able to be elucidated prior to patient expiring. 
• Case Presentation 
• A 48-year-old female with questionable past medical history of SLE and chronic inflammatory demyelinating polyneuropathy (CIDP), treated with weekly intravenous immunoglobulins (IVIG), presents to the hospital due to difficulty with activities of daily living, including eating and bathing. Family endorsed a thirty-pound weight loss with progressive dysphagia. CT Head showed right tentorium cerebellar lesion and CT abdomen and pelvis showed necrotic bilateral adrenal masses. Left retroperitoneal lymph node biopsy showed a benign reactive lymph node. Completed a Positron emission tomography (PET) scan depicting intensely avid bilateral adrenal masses with retroperitoneal and pelvic lymphadenopathy. 
• Three weeks after discharge, she presented back to hospital with worsening lethargy, microcytic anemia with hemoglobin of 6.9, hypotensive due to adrenal crisis responsive to fluids and stress dose steroids. New diffuse lower extremities purpuric lesions noted, for which biopsy showed leukocytoclastic vasculitis. Admitted to Medical Intensive Care Unit for worsening hyponatremia after fluid resuscitation and was started on 2% hypertonic saline. She was downgraded after resolution of hyponatremia and hypotension. She was again upgraded due to worsening fevers to 103F and started on broad spectrum antimicrobials: meropenem, vancomycin and fluconazole. Flow cytometry studies were negative. A large filling defect in the suprarenal inferior vena cava was noted and was started on apixaban. Despite all measures, the patient ultimately developed worsening hypoxic respiratory failure, was converted to comfort care measures and ultimately expired. 
• Pathology indicated Diffuse Large B-Cell Lymphoma returned after the patient expired. Initial differential diagnoses for this patient included lymphoma with negative biopsy due to steroid use as outpatient, Castleman disease, Rosai Dorfman disease or unspecified autoimmune disease. 
• Discussion 
• This case involves a young woman with controlled neurologic disease and multiple rheumatologic diagnoses. She presented with bilateral necrotic adrenal masses leading to adrenal insufficiency, which complicated her condition. Despite undergoing biopsy, no single diagnosis or syndrome was initially identified to explain the constellation of symptoms, and 
• unfortunately, the patient passed away from acute hypoxic respiratory failure. Diagnosis was limited by unusual symptom presentation and negative first biopsy. 



A Fatal case of Diffuse Large B Cell Lymphoma Presenting with Bilateral Necrotic Renal Masses

Background: Moyamoya disease is a rare, progressive vasculopathy that causes narrowing of intracranial arteries such as distal branches of the internal carotid artery (ICA) and proximal components of the middle anterior artery (MCA) and anterior cerebral artery (ACA). One of the ICA branches involved in Moyamoya syndrome include the anterior choroidal artery, which may develop an aneurysm, or more rarely like in this case, a pseudoaneurysm that may rupture and lead to a hemorrhagic stroke. 

Case Presentation: The patient is a 24 year-old-woman with a past medical history of sickle cell disease who presented to our neighboring hospital with left sided numbness. The patient reported an associated headache, nausea, and vomiting prior to the episode that prompted her to seek medical attention. She was transferred to our hospital due to a basal ganglia hemorrhage found on CT. Upon arrival at our emergency department, her vitals were stable and she was somnolent but AAOx3. Neurological exam was remarkable for left side hemiparesthesia and associated mild left side hemiparesis with motor strength of 4/5 in left upper and lower extremities. Patient was admitted to ICU level of care and neurology and neurointerventional radiology were consulted for further management. Brain MRI was remarkable for right thalamic intra-axial hematoma. Subsequent brain MRA was recommended and showed occluded bilateral ACA and MCA suspicious for Moyamoya syndrome. Digital subtraction angiography (DSA) was then performed showing a 4mm pseudoaneurysm of the right distal anterior choroidal artery which underwent successful embolization.

Discussion: The association between Moyamoya syndrome and sickle cell disease in the pediatric population has been well established, however, current literature is limited in regard to this disease in the adult population. Although other reports have described distal anterior choroidal artery aneurysms in Moyamoya syndrome, seldom to none have described a pseudoaneurysm of this artery in the context of Moyamoya syndrome and Sickle cell disease. Thus, our case report highlights an uncommon finding of Moyamoya syndrome whilst raising awareness of Moyamoya syndrome in adults with sickle cell anemia.

A Rare Anterior Choroidal Artery Pseudoaneurysm Secondary to Moyamoya Syndrome in a Patient with Sickle Cell Anemia

Background

Burkitt lymphoma is a highly aggressive form of non-Hodgkin lymphoma, with an increased risk associated with Human Immunodeficiency Virus (HIV) infection. In the United States, Burkitt lymphoma often involves intra-abdominal organs or lymph nodes. Here, we present an interesting and exceedingly rare case of Burkitt lymphoma involving the heart in an HIV patient.

Case Presentation

A 40-year-old male presented with a newly noticed swelling in his right axilla. He reported a 40-pound weight loss along with occasional fever, night sweats, and abdominal pain. Physical examination revealed a 4 cm subcutaneous mass without skin changes. Vital signs and initial lab results were unremarkable. CT scans of the chest, abdomen, and pelvis revealed a 7-cm right axillary mass, marked diffuse thickening of the gastric and small bowel walls, and a large right atrial filling defect.
An excisional biopsy of the axillary mass confirmed Burkitt lymphoma. HIV serology was positive, with a viral load of 229,000 copies/ml, and LDH was elevated at 503 U/L. Peripheral blood smear showed no atypical cells, and bone marrow aspiration, biopsy, and flow cytometry were negative for lymphoma involvement. The patient was diagnosed with stage IIIBE Burkitt lymphoma and started on chemotherapy, including cyclophosphamide, doxorubicin, vincristine, methotrexate, ifosfamide, etoposide, and cytarabine.

After one month, a follow-up CT scan showed a significant reduction in the size of the right axillary and atrial masses, as well as in the thickening of the small bowel and gastric walls. Given the significant treatment response of the intracardiac mass, the diagnosis was confirmed as Burkitt lymphoma with intracardiac involvement.

Discussion

Intracardiac Burkitt lymphoma is an extremely rare malignancy, with only 22 reported cases to date. As with other forms of Burkitt lymphoma, HIV infection increases the risk of developing intracardiac Burkitt lymphoma. The symptoms are often nonspecific and cardiopulmonary symptoms such as palpitations or dyspnea can occur in other types of Burkitt lymphoma as well.

Given the highly aggressive nature of intracardiac Burkitt lymphoma and its potential to cause arrhythmias or pericardial effusion, which can lead to life-threatening conditions, early diagnosis is crucial. However, the extreme rarity of the disease and the nonspecific nature of its symptoms often lead to a low level of awareness among physicians, increasing the likelihood of missed diagnoses. Therefore, it is essential for physicians to maintain a high index of suspicion for intracardiac involvement when diagnosing Burkitt lymphoma.


A Rare Case of Burkitt Lymphoma Intracardiac Involvement in an HIV Patient

Abstract Title: A Rare Find: Diagnosis and Prophylactic Treatment of Factor VII Deficiency in a Pregnant Female Undergoing Cesarian Section 

Background: 
Factor VII deficiency is a rare inherited autosomal recessive bleeding disorder which can vary greatly in its presentation and management. Factor VII plays a vital role in secondary hemostasis through its interaction with tissue factor in the extrinsic coagulation pathway activated in times of vascular injury. Phenotype can range from patients being asymptomatic to developing life-threatening bleeding involving the brain or gastrointestinal tract. Diagnosis is suspected in a bleeding patient with an isolated prolongation of the prothrombin time and confirmed with a serum factor VII level. Treatment consists of recombinant factor VIIa replacement therapy with the number of recommended doses dependent on the severity of bleeding. 

Case Presentation: 
Our patient is a 32-year-old Hispanic female who presented at 37 weeks pregnancy for planned Cesarean and further evaluation of abnormal coagulation studies noted at an outside facility. She endorsed symptoms of easy bruising, bleeding gums with oral hygiene, occasional nosebleeds and an extensive history of heavy menstrual bleeding.  She had previously been hospitalized for blood transfusion five years earlier for heavy menstrual bleeding lasting one month.  Family history was significant for a sister who had recently been diagnosed with a coagulopathy though patient was unsure of her sister's diagnosis.   Lab work showed a prothrombin time (PT) greater than 169 seconds, INR >15, PTT 25.7 seconds and complete blood count demonstrated hematocrit 35% and platelet count of 145,000.  She was given one dose of oral vitamin K 10 mg without benefit.  Factor VII level was found to be less than 0.4 and PT correction was noted with one-to-one mix, confirming a diagnosis of factor VII deficiency. Initial dose of recombinant VIIa 20 mcg/kg was given before her planned C-section with the next dose at 15 mcg/kg 4 hours post-operatively and every 6 hours thereafter for a total of 5 doses in a 24-hour period. She underwent cesarean section without significant bleeding and her PT and INR normalized with the therapy outlined above. She was discharged from the hospital and at 1 month follow-up had no further episodes of bleeding. 

Discussion: 
Our patient received factor VII replacement therapy and did not experience excessive bleeding with Cesarean section. This case demonstrates the phenotypic variation in the potential bleeding associated with Factor VII deficiency, with one-third of patients being asymptomatic during their life. Factor VII deficiency remains a disease to keep on one’s differential for an isolated elevation in PT and INR with a significant personal or family history of bleeding.

A Rare Find: Diagnosis and Prophylactic Treatment of Factor VII Deficiency in a Pregnant Female Undergoing Cesarian Section

Introduction
Castleman disease, a rare lymphoproliferative disorder, is classified as unicentric (one lymph node) or multicentric (multiple regions). The latter include POEMS-associated, HHV-8-associated, and idiopathic type . POEMS syndrome features polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Approximately about 15 % of POEMS patients may have Castleman disease.
Case Presentation
A 28-year-old male from North Kerala, India, presented with pleuritic chest pain, worsening dyspnea, abdominal distention, intermittent fever, weight loss, and loss of appetite over months. He had a history of treated symmetrical polyarthritis. Examination revealed palpable bilateral lymph nodes, low normal BP, tachycardia, tachypnea, left tracheal deviation, stony dullness on lung percussion, and absent breath sounds bilaterally. Abdomen showed mild distention, palpable liver 2 cm below the costal margin, and dullness over Traube’s space. Neurological and musculoskeletal exams were unremarkable except for reduced CMAP amplitude in bilateral peroneal nerves on nerve conduction studies.
Laboratory findings included positive ANA and Anti-TPO antibodies, suggesting autoimmune etiology. Liver function showed albumin-globulin reversal indicating chronic inflammation. Negative HHV-8 ruled out multicentric Castleman disease. Serum Immunoelectrophoresis showed normal pattern, but elevated kappa lambda light chain ratio (1.86). Elevated ferritin (572 ng/mL) and LDH (752 U/L) indicated cellular damage or inflammation.
Pleural fluid analysis revealed exudative nature (4844 cells/cubic cm, pleural protein 4.1 g/dL, LDH 312 U/L). AFB and CBNAAT tests negative ruled out tuberculosis. ADA levels normal, cultures negative for bacterial infection. Cytology negative for malignancy; serology negative for HIV and other viruses.
Imaging showed bilateral pleural effusion, lower lobe collapse, moderate pericardial effusion, and mild interstitial thickening.
Excision biopsy of the right axillary lymph node showed features consistent with Castleman disease, specifically the hyaline vascular variant. Histopathological findings included distorted lymph node architecture, increased lymphoid follicles, atretic follicles, and sclerotic blood vessels penetrating the germinal centers (lollipop lesions). Mantle zones displayed concentric rings of small lymphocytes (onion skin arrangement).
Discussion
The diagnosis of POEMS syndrome in our patient is supported by the mandatory criteria of polyneuropathy and monoclonal gammopathy, confirmed by an abnormal light chain ratio. Additionally, Castleman disease fulfills a major criterion, while minor criteria such as pleural effusion, hepatomegaly, and thyroid endocrinopathy further contribute to the diagnosis.The patient was started on pulse corticosteroid therapy and low dose immunosuppressants that brought the patient into remission. This case highlights the importance of a comprehensive diagnostic approach and multidisciplinary management in addressing Castleman disease, hyaline vascular variant, with potential concurrent POEMS syndrome

A Tale of two syndromes: The Intersection of Castleman disease and POEMS Syndrome

Background 
Renal cell carcinoma (RCC) is the most prevalent kidney malignancy in adults, accounting for 80-85% of primary renal tumors. In advanced-stage RCC, up to 30% of patients develop skeletal metastasis, causing severe bone pain and reduced quality of life. Bone metastases are often due to increased blood flow and red marrow density in highly vascularized long and flat bones. While acrometastasis—metastasis to distal bones—is rare, the high vascularity and marrow density in adult hand bones may contribute to its occurrence in 0.1-0.3% of cases. This report describes a case where RCC recurrence was indicated by left hand acrometastasis one year after initial malignancy diagnosis. 

Case Presentation 
A 65-year-old man with a history of renal cell carcinoma presented with persistent pain and swelling in the distal left long finger for 6 months. Examination revealed tenderness, swelling, and erythema in the distal phalanx, but no issues in the shoulder, elbow, or forearm. An incisional biopsy confirmed metastatic RCC to the distal phalanx, with X-rays showing a destructive process and MRI revealing an enhancing subcutaneous mass with mild erosive changes. Orthopedic consultation led to a distal interphalangeal joint disarticulation of the finger. Post-surgery pathology confirmed metastatic RCC, and the patient was later admitted to hospice care. 

Discussion 
Acrometastasis in RCC results from hematogenous rather than lymphatic spread. Cancer cells must survive in the systemic circulation, invade the sinusoidal vessels, and establish a blood supply in the bone marrow to initiate metastasis. Key factors include heparanase, which promotes metastasis by degrading extracellular matrix components, and RANKL, which facilitates cancer cell migration and osteoclastogenesis. Both RANKL and its receptor RANK, as well as the decoy receptor OPG, are involved in bone remodeling and metastatic processes. 

Clinical signs of acrometastasis include erythema, pain, swelling, and functional impairment, but can mimic conditions like osteomyelitis or gout. Early and accurate diagnosis is crucial, necessitating imaging studies and tissue biopsies when possible. Distinguishing acrometastasis from other conditions is essential for appropriate treatment, given its poor prognosis with a median survival of 6 months. 

The rarity of acrometastasis means there is no standard treatment protocol; management focuses on palliation, including pain control, resection, and maintaining function. Resection might involve ray amputation or more conservative approaches like radiation for large, inoperable lesions. Though prognosis is generally poor, solitary osseous RCC lesions may have a better outcome with wide resection and nephrectomy.

Acrometastatic Disease of the Hand as an Indicator of Renal Cell Carcinoma Recurrence

Background
Ketorolac (Toradol) is a non-steroidal anti-inflammatory drug (NSAID) that is commonly used to reduce pain and inflammation. It inhibits prostaglandin synthesis by competitively blocking cyclooxygenase (COX) enzymes. Although Toradol has known adverse side effects to include agranulocytosis, cases of such are extremely rare. Here we present a unique case of severe agranulocytosis following Toradol administration.
Case Presentation
A 72 year-old male with a past medical history of moderate-severe rheumatoid arthritis on methotrexate, golimumab (Simponi) and prednisone, hypertension and type two diabetes mellitus presented with one week of progressive oropharyngeal dysphagia and odynophagia consistent with severe esophageal candidiasis; he was admitted for treatment with fluconazole. In the 2 days prior to admission, the patient was seen in the emergency room setting on multiple occasions and was administered 75 mg of intramuscular Toradol. His complete blood count (CBC) the day prior to admission was significant for a normal WBC (6.81), a mild anemia (11.5), and a mild thrombocytopenia (145). On the day of admission he received an additional 30 mg of Toradol. The following CBC revealed pancytopenia with a leukopenia of 0.72, anemia of 10.4, and thrombocytopenia of 101. Toradol was discontinued, and his home regimen for rheumatoid arthritis was held the entirety of admission. A broad workup for etiologies of pancytopenia was unrevealing. Viral serologies were negative, hemolytic labs and blood smear showed no signs of hemolysis as well as no blasts. Felty syndrome was also considered but imaging did not show splenomegaly. Additionally, an iron panel, B12 and Folate were normal. Bone marrow suppression was evident by a reticulocyte of 0.27% and a rapid drop in WBC and thus the patient was initiated on Neupogen, a granulocyte colony-stimulating factor, and leucovorin. Bone marrow biopsy and flow cytometry revealed hypercellular/hyperproliferative myeloid elements and low level of blasts (5%) after G-CSF administration. The patient required platelet and blood transfusions on hospital day 6 and counts began to recover shortly after. Patient was discharged after cell lines had returned to safe levels.
Discussion
In patients who are unable to tolerate oral NSAIDs, Toradol is commonly used. While
cytopenias are often seen in the in-patient setting, providers should be aware of potential side effects of NSAIDs including the rarely-seen severe agranulocytosis to allow for early discontinuation of offending medications.

Agranulocytosis Following Ketorolac Administration

Introduction:

Light chain (AL) amyloidosis is a rare plasma cell dyscrasia characterized by extracellular deposition of light chains in various organs, showing distinctive Congo Red (CR) staining on involved organ biopsy. Most patients with plasma cell dyscrasias will have evidence of a monoclonal protein in the serum or urine but it can be absent in approximately 3% of patients. For such patients, serum free light chain (FLC) assay is a critical component of diagnostic evaluation. Amyloidosis can have broad clinical findings with non-specific manifestations including cutaneous, musculoskeletal, and cardiac, that overlap with certain rheumatological disorders like scleroderma. This makes amyloidosis a challenging diagnosis, which is often diagnosed after significant delay or misdiagnosed as another entity. We present a case of amyloidosis initially misdiagnosed as scleroderma. 

Case presentation: 

A 54-year-old woman with a past medical history of ulcerative colitis and rectal adenocarcinoma treated with surgical resection presented with severe skin thickening, pruritus, macroglossia, Raynaud’s phenomenon, and inflammatory arthritis. An extensive serologic workup for rheumatologic conditions was negative for a definitive diagnosis. As part of the workup, serum protein electrophoresis (SPEP) was obtained which showed hypogammaglobulinemia, however, no immunofixation (IFE) or FLC assay was ordered. Despite the negative serologic workup, due to high clinical suspicion, she was treated for a presumed diagnosis of scleroderma with sequential trials of prednisone, methotrexate, and mycophenolate mofetil, without improvement in her symptoms. About 2 years later with macroglossia still present, repeat SPEP, IFE, and FLC were obtained and revealed no M spike but did show lambda light chains elevated to 36.58 mg/dL, kappa light chains 0.5 mg/dL, with a FLC ratio of 73 (normal 0.26 to 1.65). Subsequently, a bone marrow (BM) biopsy was performed, which showed mildly hypocellular BM for her age (30% cellularity) and an abnormal plasma cell population constituting approximately 30% of the marrow, identified by immunohistochemistry against CD138, and they were found to be lambda light chain restricted by in-situ hybridization. CR stain was negative on the BM biopsy. Due to suspicion of underlying amyloidosis given history of macroglossia, an echocardiogram was ordered, which revealed moderate concentric hypertrophy of the left ventricle, consistent with infiltrative cardiomyopathy, which was then confirmed on cardiac MRI. An endomyocardial biopsy was subsequently performed, which stained positive for CR, and liquid chromatography/mass spectrometry confirmed AL amyloidosis. The patient was treated with amyloidosis directed therapies followed by an autologous peripheral blood stem cell transplant. Her prior symptoms resolved after treatment and macroglossia improved by over 50%.


Discussion: 

Amyloidosis may initially present with non-specific manifestations like skin thickening and infiltrative cardiomyopathy that can have clinical overlap with various rheumatologic disorders such as scleroderma. Our patient was initially misdiagnosed with scleroderma. Increased awareness and checking FLC may have led to earlier diagnosis and improvement in morbidity. This case highlights the need to consider an alternative diagnosis when symptoms fail to respond to appropriate therapy and the need for provider awareness regarding complete workup for paraproteinemia including FLC with SPEP and IFE when looking for amyloidosis as 50% of amyloidosis cases have light chain only involvement. 


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