scholarly journals Clinical spectrum of POEMS-associated multicentric Castleman disease with renal involvement: a diagnostic challenge

2019 ◽  
Vol 8 (2) ◽  
pp. 21-21
Author(s):  
Caroline Kullmann Ribeiro ◽  
Fernanda Bresciani ◽  
Samile Echeverria Silveira ◽  
Pedro Guilherme Schaefer ◽  
Elvino Guardão Barros ◽  
...  
Keyword(s):  
Renal Function ◽  
Lymph Node ◽  
Plasma Cells ◽  
Clinical Laboratory ◽  
Renal Involvement ◽  
Case Series ◽  
Human Herpesvirus ◽  
Castleman Disease ◽  
Diagnostic Challenge ◽  
Multicentric Castleman Disease

Background: Castleman disease (CD) is a rare and heterogeneous lymphoproliferative disorder with a wide variety of clinical presentations and outcomes. Human herpesvirus-8 (HHV-8) related CD corresponds to the most common subtype of the multicentric Castleman disease (MCD). However, if HHV-8 is negative, POEMS (peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) associated with MCD or idiopathic MCD are the cause in a subgroup of patients. Considering the rarity of POEMS and MCD association, we herein describe a patient with a typical presentation based on clinical, laboratory and tissue biopsy data. Case Presentation: We report a diabetic patient who presented with asthenia, edema, skin lesions manifested by scarring in chiropodactyls, multiple lymph node enlargement in the neck, armpits and inguinal areas, splenomegaly, severe anemia, thrombocytopenia, and mixed polyneuropathy. Hematuria and proteinuria were detected. The patient developed progressive renal failure requiring dialysis. Renal biopsy showed mesangial expansion with mesangial hypercellularity, and lymphoplasmacytoid cells focally distributed in tubules and interstitium, which were compatible with acute tubulointerstitial nephritis. In immunofluorescence, no deposits of IgG, IgA, IgM, C1q, C3 or fibrinogen were found, and kappa and lambda were also negative. Lymph node biopsy revealed lymphoid tissue with follicular hyperplasia, sinusoidal and medullary infiltration of plasma cells. Immunohistochemistry confirmed positivity for B lymphocytes, T lymphocytes, and plasma cells in sub-capsular and para-follicular areas. The patient was diagnosed as POEMS-associated MCD variant, and chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone was started. The patient did not recover renal function and remained dialysis-dependent. Conclusions: To date, the renal involvement in MCD and POEMS syndrome seems to be uncommon as reported in few case series. Its pathophysiology is not well understood. In the spectrum of MCD, decreased renal function may have impact in patient survival. Early diagnosis and treatment are needed to control the systemic manifestations, and most importantly to avoid chronic organ damage.

scholarly journals Kidney biopsy findings in two patients with TAFRO syndrome: case presentations and review of the literature

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Qianyun Zhou ◽  
Yuanyuan Zhang ◽  
Guangping Zhou ◽  
Jihong Zhu
Keyword(s):  
Lymph Node ◽  
Renal Insufficiency ◽  
Epigastric Pain ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Castleman Disease ◽  
Tafro Syndrome ◽  
Multicentric Castleman Disease ◽  
Renal Biopsies ◽  
Case Presentations

Abstract Background TAFRO syndrome is a clinical subtype of idiopathic multicentric Castleman disease (iMCD) that is characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis (or renal dysfunction), and organomegaly. TAFRO syndrome has only recently been described, and many clinicians are unaware of this disease, leading to delays in diagnosis and treatment. We present two patients with TAFRO syndrome in whom renal biopsies were performed. Case presentation Both patients had subacute onset and exhibited renal insufficiency, edema, anemia, thrombocytopenia, polyserositis and lymphadenopathy over the disease course. However, there were many differences in their clinical manifestations. Case 1 was a 30-year-old woman admitted due to intermittent vaginal bleeding for 3 weeks. Laboratory tests on admission showed severe renal insufficiency (creatinine: 624 μmol/L), severe anemia (Hb: 41 g/L), and moderate thrombocytopenia (61 × 109/L). Case 2 was a 42-year-old man. Acute epigastric pain was his initial complaint, and computed tomography (CT) revealed retroperitoneal exudation around the pancreas. He was diagnosed with acute pancreatitis, and after treatment with a proton pump inhibitor (PPI) and somatostatin, his abdominal pain still recurred. During treatment, renal failure gradually increased, with oliguria, fever, anemia, thrombocytopenia, edema and massive ascites. Lymph node histologies were consistent with the hyaline-vascular (HV) type and mixed type, respectively, and renal histopathologies were consistent with thrombotic microangiopathy (TMA)-like renal lesions and membranoproliferative glomerulonephritis (MPGN), respectively. Their general conditions improved after glucocorticoid therapy, but their renal functions did not recover completely. On the basis of glucocorticoids, second-line treatments with tocilizumab and rituximab, respectively, were applied. Conclusions The diagnosis of TAFRO syndrome is based mainly on clinical manifestations and lymph node biopsies. A reliable early diagnosis and appropriate rapid treatment are essential to improve patient outcomes. Clinicians should deepen their understanding of this disease and similar conditions. Once the disease is suspected, lymph node biopsies should be performed as soon as possible. In addition, renal biopsies should be actively performed in patients with renal involvement.

scholarly journals Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease

Blood ◽  
2018 ◽  
Vol 132 (22) ◽  
pp. 2323-2330 ◽  
Author(s):  
David C. Fajgenbaum
Keyword(s):  
Lymph Node ◽  
Signaling Pathways ◽  
Human Herpesvirus ◽  
Cell Types ◽  
Evidence Base ◽  
Multiple Organ ◽  
Castleman Disease ◽  
Proteomic Profiling ◽  
Durable Response ◽  
Multicentric Castleman Disease

Castleman disease (CD) describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. Patients of all ages present with either a solitary enlarged lymph node (unicentric CD) or multicentric lymphadenopathy (MCD) with systemic inflammation, cytopenias, and life-threatening multiple organ dysfunction resulting from a cytokine storm often driven by interleukin 6 (IL-6). Uncontrolled human herpesvirus-8 (HHV-8) infection causes approximately 50% of MCD cases, whereas the etiology is unknown in the remaining HHV-8-negative/idiopathic MCD cases (iMCD). The limited understanding of etiology, cell types, and signaling pathways involved in iMCD has slowed development of treatments and contributed to historically poor patient outcomes. Here, recent progress for diagnosing iMCD, characterizing etio-pathogenesis, and advancing treatments are reviewed. Several clinicopathological analyses provided the evidence base for the first-ever diagnostic criteria and revealed distinct clinical subtypes: thrombocytopenia, anasarca, fever, reticulin fibrosis/renal dysfunction, organomegaly (iMCD-TAFRO) or iMCD-not otherwise specified (iMCD-NOS), which are both observed all over the world. In 2014, the anti-IL-6 therapy siltuximab became the first iMCD treatment approved by the US Food and Drug Administration, on the basis of a 34% durable response rate; consensus guidelines recommend it as front-line therapy. Recent cytokine and proteomic profiling has revealed normal IL-6 levels in many patients with iMCD and potential alternative driver cytokines. Candidate novel genomic alterations, dysregulated cell types, and signaling pathways have also been identified as candidate therapeutic targets. RNA sequencing for viral transcripts did not reveal novel viruses, HHV-8, or other viruses pathologically associated with iMCD. Despite progress, iMCD remains poorly understood. Further efforts to elucidate etiology, pathogenesis, and treatment approaches, particularly for siltuximab-refractory patients, are needed.

scholarly journals Treatment of Unresectable Unicentric Castleman Disease with Therapeutic Embolization

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2415-2415
Author(s):  
Meera Mohan ◽  
James C Meek ◽  
Mary Elizabeth Meek ◽  
Ralph Lynn Broadwater ◽  
Katie Stone ◽  
...  
Keyword(s):  
Lymph Node ◽  
Conflicts Of Interest ◽  
Incidental Finding ◽  
Case Reports ◽  
Case Series ◽  
Human Herpesvirus ◽  
Therapeutic Embolization ◽  
Castleman Disease ◽  
Primary Therapy ◽  
Additional Surgery

Abstract Introduction: Unicentric Castleman disease (UCD) is a rare non-clonal lymphoproliferative disorder affecting one lymph node station. UCD can be an incidental finding on radiologic studies, whilst other patients have symptomatology due to compression of vital structures. Surgical extirpation is the preferred therapy and is usually curative, but unresectable UCD can represent a therapeutic challenge. Castleman lymph nodes are often highly vascularized, which offers the opportunity for therapeutic embolization. We report a series of 6 patients with unresectable UCD who were treated with embolization either as sole therapy or supplemented by cryoablation and surgery. Methods: CT rotational angiography was performed to localize the arterial supply of UCD masses. Feeding vessels were selectively embolized using a 50:50 mixture of lipiodol and alcohol or 300-500 micron embospheres. A second arteriography was performed 2 to 3 months later to identify and embolize any new arterial channels. Results: Data is summarized in Table 1. Of a cohort of 47 patients with UCD, 6 (13%) were found to have symptomatic, unresectable disease. All patients were HIV and human herpesvirus-8 negative. The median patient age was 34 years (range: 28-34); five patients were male and one was female. Disease was localized to the pelvis (n=3), mediastinum (n=2), and axilla (n=1). In all but one case, the histology was of the hyaline vascular variety. Four patients had failed R-CHOP, rituximab/steroids, or both. In 2 patients, embolization was done as primary therapy, while 3 underwent additional surgery. In 5 patients, embolization was performed twice to ablate secondary arterial channels that had appeared after the first procedure. Adjunctive cryoablation at the time of embolization was applied in 2 patients. All treated patients had major reduction in their lymph node mass. The median reduction in tumor bulk was from 274cc (range:13-969) to 21cc (range: 3-394). One patient with an axillary mass involving the brachial plexus failed therapy and received radiation. A second patient had regrowth of the UCD and responded to combination lenalidomide and obinituzumab. Responses were sustained for at least 2 years in the remaining patients. Conclusion: A small number of case reports have been described UCD patients treated with arterial embolization as an immediate preoperative adjunct to surgery to limit intraoperative bleeding. In the present series, we utilized embolic devascularization to achieve cytoreduction rather than merely prevent surgical hemorrhage. Embolization was complemented by cryoablation and rendered surgery feasible. This case series highlights that effective disease control can be obtained of unresectable UCD using a multimodality approach in which vascular embolization plays a crucial role. Disclosures No relevant conflicts of interest to declare.

scholarly journals Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease

Hematology ◽  
2018 ◽  
Vol 2018 (1) ◽  
pp. 318-325 ◽  
Author(s):  
David C. Fajgenbaum
Keyword(s):  
Lymph Node ◽  
Signaling Pathways ◽  
Human Herpesvirus ◽  
Cell Types ◽  
Evidence Base ◽  
Multiple Organ ◽  
Castleman Disease ◽  
Proteomic Profiling ◽  
Durable Response ◽  
Multicentric Castleman Disease

Abstract Castleman disease (CD) describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. Patients of all ages present with either a solitary enlarged lymph node (unicentric CD) or multicentric lymphadenopathy (MCD) with systemic inflammation, cytopenias, and life-threatening multiple organ dysfunction resulting from a cytokine storm often driven by interleukin 6 (IL-6). Uncontrolled human herpesvirus-8 (HHV-8) infection causes approximately 50% of MCD cases, whereas the etiology is unknown in the remaining HHV-8-negative/idiopathic MCD cases (iMCD). The limited understanding of etiology, cell types, and signaling pathways involved in iMCD has slowed development of treatments and contributed to historically poor patient outcomes. Here, recent progress for diagnosing iMCD, characterizing etio-pathogenesis, and advancing treatments are reviewed. Several clinicopathological analyses provided the evidence base for the first-ever diagnostic criteria and revealed distinct clinical subtypes: thrombocytopenia, anasarca, fever, reticulin fibrosis/renal dysfunction, organomegaly (iMCD-TAFRO) or iMCD-not otherwise specified (iMCD-NOS), which are both observed all over the world. In 2014, the anti-IL-6 therapy siltuximab became the first iMCD treatment approved by the US Food and Drug Administration, on the basis of a 34% durable response rate; consensus guidelines recommend it as front-line therapy. Recent cytokine and proteomic profiling has revealed normal IL-6 levels in many patients with iMCD and potential alternative driver cytokines. Candidate novel genomic alterations, dysregulated cell types, and signaling pathways have also been identified as candidate therapeutic targets. RNA sequencing for viral transcripts did not reveal novel viruses, HHV-8, or other viruses pathologically associated with iMCD. Despite progress, iMCD remains poorly understood. Further efforts to elucidate etiology, pathogenesis, and treatment approaches, particularly for siltuximab-refractory patients, are needed.

scholarly journals Pi3k/Akt/Mtor Pathway Activity Is Increased in Lymph Node Tissue from Idiopathic Multicentric Castleman Disease Patients with Tafro Syndrome

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1121-1121
Author(s):  
Dustin Shilling ◽  
Dale M Kobrin ◽  
David C Fajgenbaum
Keyword(s):  
Lymph Node ◽  
Lymph Nodes ◽  
Signaling Pathways ◽  
Germinal Center ◽  
Plasma Cells ◽  
Human Herpesvirus ◽  
Castleman Disease ◽  
Mtor Signaling ◽  
Breast Cancer Patients ◽  
Color Deconvolution

Abstract Castleman disease (CD) describes a group of heterogeneous diseases defined by shared lymph node histopathology, including atrophic or hyperplastic germinal centers, prominent follicular dendritic cells, hypervascularization, polyclonal lymphoproliferation, and/or polytypic plasmacytosis. Unicentric CD (UCD) involves a solitary enlarged lymph node that displays CD histopathology, and patients rarely experience systemic symptoms. In contrast, multicentric CD (MCD) involves multiple regions of enlarged lymph nodes, systemic inflammation, cytopenias, and vital organ dysfunction due to a cytokine storm often including interleukin-6. MCD is caused by uncontrolled infection with Kaposi sarcoma-associated/human herpesvirus-8 (HHV-8) in ~50% of cases. The etiology of the remaining HHV-8-negative MCD cases is idiopathic (iMCD). In iMCD patients, blockade of IL-6 signaling with siltuximab, the only FDA-approved iMCD treatment, induced responses in 34% of cases in the phase II registrational trial. The large proportion of non-responders suggest that alternative pathways are responsible for driving disease pathogenesis in some patients. For these individuals, identification of molecular and cellular abnormalities for therapeutic targeting is urgently needed, particularly for those with the most severe clinical presentations. In fact, a clinical subgroup of iMCD was recently described with a very severe presentation: thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly (iMCD-TAFRO). We previously reported increased phosphoinositide 3-kinase (PI3K)/Akt/mechanistic target of rapamycin (mTOR) signaling--a central pathway downstream of multiple cell surface receptors, implicated in both autoimmune and oncologic disorders--in a treatment refractory iMCD-TAFRO case that experienced an extended remission on treatment with an mTOR inhibitor. To extend these findings, herein we report immunohistochemistry for phosphorylated ribosomal protein S6 (phospho-S6), a marker of mTOR activation, in lymph node tissue from additional iMCD-TAFRO cases (n=10) and sentinel lymph nodes from breast cancer patients without evidence of metastasis (n=5). Anti-phospho-S6 (Ser235/236, Clone D57.2.2E) was used following standard protocols, and Aperio ImageScope and Image Analysis Toolkit software (color deconvolution v9 algorithm) were used to quantify pixel staining intensity in the germinal center, mantle zone, follicular and interfollicular regions. This analysis identified an increased number of pixels staining weak, medium, and strong for phospho-S6 in the interfollicular region of iMCD-TAFRO cases (p<0.005 for all comparisons) and an increased number of pixels staining weak for phospho-S6 in the germinal center (p<0.05) compared to control cases. Given that T cells are largely represented in the interfollicular region and mTOR signaling is critical to T cell proliferation, we hypothesized that the observed increase in phospho-S6 signal would occur in CD3+ cells. However, co-immunofluorescence assays for phospho-S6 and CD3 (Dako, A0452) across iMCD-TAFRO cases identified 0.08 ± 0.16% (mean ± standard deviation; n = 4 cases) of phospho-S6-positive cells as expressing CD3. In contrast, co-immunofluorescence for phospho-S6 and CD138 (Dako, Clone MI15) revealed 17.89 ± 11.26% of phospho-S6-positive cells as plasma cells. iMCD is considered an IL-6 driven disorder; however, anti-IL-6 therapy is effective in only a portion of cases. Alternative signaling pathways driving CD pathogenesis are poorly understood. This study provides the largest quantification to-date of aberrant PI3K/Akt/mTOR activity in iMCD-TAFRO, the first systematic study demonstrating increased mTOR activation in iMCD-TAFRO, and the first to identify a cell type, plasma cells. These findings are key to advancing our understanding of the pathological cell types and disrupted signaling pathways in iMCD. Disclosures Fajgenbaum: Janssen Pharmaceuticals, Inc.: Research Funding.

scholarly journals IgG4-related intracranial disease

2018 ◽  
Vol 32 (1) ◽  
pp. 29-35 ◽  
Author(s):  
Shahine Goulam-Houssein ◽  
Jeffrey L Grenville ◽  
Katerina Mastrocostas ◽  
David G Munoz ◽  
Amy Lin ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Surgical Intervention ◽  
Case Series ◽  
Hypertrophic Pachymeningitis ◽  
Diagnostic Challenge ◽  
Perineural Spread ◽  
Related Disease ◽  
The Third ◽  
Igg4 Related Disease ◽  
Intracranial Involvement

IgG4-related disease (IgG4-RD) is a multi-organ chronic inflammatory process caused by infiltration of IgG4-positive plasma cells in one or more organs. Intracranial involvement has only recently become better recognized. Our case series adds to the growing literature on the varying presentations of intracranial IgG4 by describing the clinical and imaging findings of three patients who presented to our institution with intracranial involvement. Our first patient presented with a mass-forming IgG4 pachymeningitis mimicking a sphenoid wing meningioma, which is to our knowledge the largest mass-forming pachymeningitis published in the literature. Our second case depicts another presentation of extensive IgG4 pachymeningitis involving both cavernous sinuses and surrounding Meckel’s caves. The third case describes a patient with presumed lymphocytic hypophysitis, which was later determined to be IgG4-related hypophysitis with concomitant pachymeningitis and perineural spread along the optic nerves. The delayed diagnoses in our cases illustrates the diagnostic challenge that clinicians face in differentiating intracranial IgG4-RD from other infiltrative diseases such as sarcoidosis, granulomatous disease, tuberculosis and lymphoma. Earlier consideration of IgG4-related hypophysitis and hypertrophic pachymeningitis in the differential diagnosis can prevent significant morbidity including unnecessary surgical intervention and organ failure secondary to extensive fibrosis.

scholarly journals A Review of Genetic Abnormalities in Unicentric and Multicentric Castleman Disease

Biology ◽  
2021 ◽  
Vol 10 (4) ◽  
pp. 251
Author(s):  
Alexandra Butzmann ◽  
Jyoti Kumar ◽  
Kaushik Sridhar ◽  
Sumanth Gollapudi ◽  
Robert S. Ohgami
Keyword(s):  
Plasma Cells ◽  
Treatment Options ◽  
Disease Process ◽  
Castleman Disease ◽  
Mitogen Activated Protein Kinase ◽  
Human Herpes Virus 8 ◽  
Multicentric Castleman Disease ◽  
Molecular Abnormalities ◽  
Genetic Abnormalities ◽  
Mitogen Activated Protein

Castleman disease (CD) is a rare lymphoproliferative disorder known to represent at least four distinct clinicopathologic subtypes. Large advancements in our clinical and histopathologic description of these diverse diseases have been made, resulting in subtyping based on number of enlarged lymph nodes (unicentric versus multicentric), according to viral infection by human herpes virus 8 (HHV-8) and human immunodeficiency virus (HIV), and with relation to clonal plasma cells (POEMS). In recent years, significant molecular and genetic abnormalities associated with CD have been described. However, we continue to lack a foundational understanding of the biological mechanisms driving this disease process. Here, we review all cases of CD with molecular abnormalities described in the literature to date, and correlate cytogenetic, molecular, and genetic abnormalities with disease subtypes and phenotypes. Our review notes complex karyotypes in subsets of cases, specific mutations in PDGFRB N666S in 10% of unicentric CD (UCD) and NCOA4 L261F in 23% of idiopathic multicentric CD (iMCD) cases. Genes affecting chromatin organization and abnormalities in methylation are seen more commonly in iMCD while abnormalities within the mitogen-activated protein kinase (MAPK) and interleukin signaling pathways are more frequent in UCD. Interestingly, there is a paucity of genetic studies evaluating HHV-8 positive multicentric CD (HHV-8+ MCD) and POEMS-associated CD. Our comprehensive review of genetic and molecular abnormalities in CD identifies subtype-specific and novel pathways which may allow for more targeted treatment options and unique biologic therapies.

Five biopsies, one diagnosis: challenges in idiopathic multicentric Castleman disease

2020 ◽  
Vol 13 (11) ◽  
pp. e236654
Author(s):  
Julie Semenchuk ◽  
Asad Merchant ◽  
Ali Sakhdari ◽  
Vishal Kukreti
Keyword(s):  
Lymph Node ◽  
Kidney Injury ◽  
Lymph Node Biopsy ◽  
Castleman Disease ◽  
Pleuritic Chest Pain ◽  
Multicentric Castleman Disease ◽  
Node Biopsy ◽  
Significant Clinical Improvement ◽  
Night Sweats ◽  
Inflammatory Changes

A previously healthy 29-year-old man initially presented to the hospital with pleuritic chest pain and shortness of breath. Over the next 2 months he developed ongoing fevers and night sweats with recurrent exudative pleural effusions and ascites. He had an extensive infectious and autoimmune workup that was unremarkable. He had an initial lymph node biopsy that showed reactive changes only. He had an acute kidney injury and his renal biopsy revealed thrombotic microangiopathy. His liver biopsy showed non-specific inflammatory changes. His bone marrow biopsy showed megakaryocyte hyperplasia and fibrosis, which raised suspicion for the thrombocytopenia, ascites, reticulin fibrosis, renal dysfunction and organomegaly syndrome subtype of multicentric Castleman disease. This prompted a repeat lymph node biopsy, showing changes consistent with mixed type Castleman disease that fit with his clinical picture. He was initiated on steroids and siltuximab with significant clinical improvement.

Human Herpesvirus Type 8-positive Multicentric Castleman Disease

2016 ◽  
Vol 16 ◽  
pp. S159-S165 ◽  
Author(s):  
Anait L. Melikyan ◽  
Elena K. Egorova ◽  
Hunan L. Julhakyan ◽  
Alla L. Kovrigina ◽  
Valeriy G. Savchenko
Keyword(s):  
Human Herpesvirus ◽  
Castleman Disease ◽  
Herpesvirus Type ◽  
Multicentric Castleman Disease ◽  
Human Herpesvirus Type

scholarly journals A case series of Kimura’s disease: a diagnostic challenge

2018 ◽  
Vol 9 (7) ◽  
pp. 207-211 ◽  
Author(s):  
Vivek Kumar ◽  
Navneet Mittal ◽  
Yiwu Huang ◽  
Jasminka Balderracchi ◽  
Huo Xiang Zheng ◽  
...  
Keyword(s):  
Lymph Node ◽  
Lymph Nodes ◽  
Hodgkin's Disease ◽  
Immunoglobulin E ◽  
Hodgkin’S Disease ◽  
Case Series ◽  
Kimura’S Disease ◽  
Pathological Features ◽  
Diagnostic Challenge ◽  
First Case

Kimura’s disease (KD) is a rare, benign disorder characterized by subcutaneous masses with regional lymph-node enlargement. It is considered to be due to chronic inflammation of unclear etiology. Most cases have been reported in young, 20–30-year-old men of Asian descent. The diagnosis of KD is based on pathological features and elevated immunoglobulin E levels. Characteristic pathological features include intact lymph-node architecture, florid germinal center hyperplasia, extensive eosinophilic infiltrates, and proliferation of postcapillary venules. However, these features can also be seen in Hodgkin’s disease or T-cell lymphoma, therefore, cases presenting as KD pose a diagnostic challenge. We report a case series of two cases with suspected KD at initial presentation, with one patient eventually diagnosed with Hodgkin’s disease after clinical progression. The first case was a 45-year-old Asian man who presented with bilateral thigh masses and significantly enlarged inguinal lymph nodes. The histopathology was characteristic and the patient had stable disease on treatment with cetirizine for 20 months. The second case was a 29-year-old African-American man who had progressive enlargement of the right neck lymph nodes extending into the mediastinum, with the original biopsy suggestive of KD. An initial search for Reed–Sternberg cells using immunohistochemical staining for CD15 and CD30 was negative. However, the patient developed neurological symptoms corresponding to tumor extension to the cervical and thoracic neural foramina. A repeat biopsy showed a lack of nodal structure and atypical large cells that were positive for CD30 staining. The patient was treated with chemotherapy with good response. We emphasize the importance of following the clinical course to render an accurate diagnosis. Both cases showed extensive eosinophilic infiltration and other KD-like pathological features. However, KD is rare; not missing a malignant diagnosis lies in high clinical suspicion and repeated exhaustive work up.

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