Clinical and pathological characteristics of HIV- and HHV-8–negative Castleman disease

Yu, Li; Tu, Meifeng; Cortes, Jorge; Xu-Monette, Zijun Y.; Miranda, Roberto N.; Zhang, Jun; Orłowski, Robert Z.; Neelapu, Sattva S.; Boddu, Prajwal; Akosile, Mary; Uldrick, Thomas S.; Yarchoan, Robert; Medeiros, L. Jeffrey; Li, Yong; Fajgenbaum, David C; Young, Ken H.

doi:10.1182/blood-2016-11-748855

Cited by 134 publications

(172 citation statements)

References 40 publications

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“…Typical imaging features in our cohort include nodal masses that appear uniformly hypoechoic on ultrasound and homogeneously hyperenhancing on CT and MRI. By FDG‐PET, lesions showed variable, mild to moderate avidity, which has also been shown in adult studies . Of note, UCD patients tended to have larger lesions than MCD patients.…”

Section: Discussionsupporting

confidence: 66%

“…Although laboratory inflammation is expected among MCD patients, our UCD population appeared to have increased inflammation compared with adult cohorts. For example, whereas in our cohort, 45% of UCD patients had an elevated ESR, in an adult UCD cohort this rate was 19%, and 50% had an elevated CRP …”

Section: Discussioncontrasting

confidence: 64%

“…Similarly, in our pediatric/AYA cohort, systemic symptoms were more common among MCD patients than UCD patients, although this difference in presentations by centricity is less striking than in adults. For example, 44% presented with systemic symptoms, which is above the upper range of the reported 10–40% rate of systemic symptoms reported in adult UCD reports . Among HHV8‐negative MCD patients in this cohort, four and six (67%) had systemic symptoms, which is at the lower end of the 60–80% range reported in adult cohorts …”

Section: Discussionmentioning

confidence: 44%

“…In adults, histopathologic variant is associated with variation in presentation. Adults with the HV variant are likely to have UCD and often lack systemic or constitutional symptoms; when adults with HV variant present with MCD, it is often associated TAFRO and inflammatory milieu . In contrast, patients with the PCV, regardless of centricity, often have fevers, diaphoresis, arthralgia, neuropathies, and laboratory derangements such as elevated IL‐6, hypoalbuminemia, microcytic anemia, elevated ESR, amyloidosis, and association with HHV8 .…”

Section: Discussionmentioning

confidence: 99%

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Castleman disease in pediatrics: Insights on presentation, treatment, and outcomes from a two‐site retrospective cohort study

Sopfe

Endres

Campbell

et al. 2019

Pediatric Blood & Cancer

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Background Castleman disease (CD) is an uncommon lymphoproliferative disorder that is rare in pediatric populations; the literature describing this population is sparse. We sought to describe pediatric CD, including unicentric CD (UCD) and human herpes virus‐8 (HHV8)‐negative multicentric CD (MCD), in a multi‐institutional cohort. Methods We retrospectively reviewed 24 patients, aged 0 to 26 years at diagnosis, who were diagnosed with CD between January 1, 2005, and May 16, 2017, at two tertiary children's hospitals. Demographic and clinical data were collected. Results Most patients (75%, 18/24) presented with UCD. All patients with MCD were HHV8‐negative. The most common histopathologic variant was hyaline vascular (75%, 18/24). Plasma cell variant occurred in 33% (2/6 [95% confidence intervals (CI), 4–78%]) of patients with HHV8‐negative MCD and 17% (3/18 [95% CI, 4–41%]) of patients with UCD. Systemic symptoms were present in 4 of 6 of patients with HHV8‐negative MCD and 8 of 18 of patients with UCD. Anemia and laboratory inflammation occurred in both UCD and MCD patients, with nonsignificantly higher rates of anemia and elevated C‐reactive protein in MCD patients. All but two UCD patients underwent gross total resection as definitive therapy. Among HHV8‐negative MCD patients, a combination of resection, chemotherapy, and immunotherapy was used. No UCD patients and three of six HHV8‐negative MCD patients experienced disease progression/relapse prior to lasting remission. There were no deaths. Conclusion Pediatric patients with CD most commonly have unicentric, hyaline vascular variant disease. Pediatric patients with both UCD and MCD commonly have systemic inflammation and, despite risk of progression/relapse in MCD patients, ultimately have excellent survival.

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Section: Discussionsupporting

confidence: 66%

Section: Discussioncontrasting

confidence: 64%

Section: Discussionmentioning

confidence: 44%

Section: Discussionmentioning

confidence: 99%

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Castleman disease in pediatrics: Insights on presentation, treatment, and outcomes from a two‐site retrospective cohort study

Sopfe

Endres

Campbell

et al. 2019

Pediatric Blood & Cancer

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“…It cannot be given for long period of time because its side effects [9-11]. Rituximab is another common drug used to treat iMCD, in one report describing 25 cases of iMCD treated with rituximab as first-line therapy 20% achieved CR and 48% PR [12]. In another report that described 8 patients treated with this drug alone, 5 patients achieved CR, and 1 patient had disease progression [13].…”

Section: Idiopathic Multicentric Castleman Diseasementioning

confidence: 99%

Role of Allogeneic Stem Cell Transplant in Chronic Lymphocytic Leukemia and its Implications in Current Era

Socola¹

2017

J Leuk

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An Abnormal Neck Mass in a Pediatric Patient

Boochoon

Barbieri

Mohyuddin

2020

JAMA Otolaryngol Head Neck Surg

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A 17-year-old white male patient was referred to the otolaryngology clinic with a 6-month history of a left-sided neck mass. His primary care physician initially obtained an ultrasonographic image, which demonstrated a left, level 2 lymph node measuring 4.0 × 1.5 × 2.8 cm. The patient then underwent several courses of antibiotic treatment, but the mass persisted. After a period of conservative management, he developed a sore throat and fever. He subsequently underwent a computed tomographic scan of the neck and chest with contrast, which demonstrated an isolated, enlarged, homogenously enhancing left-neck level 2B lymph node measuring 3.4 × 2.8 × 2.1 cm (Figure 1A). When he presented to our facility, he was found to have an asymptomatically enlarged left level 2/5A neck mass that was nontender and fixed. The patient had an unremarkable medical, surgical, and social history and no known infectious exposures. A white blood cell count and C-reactive protein level were both elevated, at 11 310 cells/μL (to convert to cells × 10 9 per liter, multiply by 0.001) and 56.2 mg/L (to convert to nanomoles per liter, multiply by 9.524), respectively. Studies for influenza A and B, HIV, cytomegalovirus, American foulbrood, Bartonella henselae, infectious mononucleosis, and Epstein-Barr virus had negative results. An ultrasonographyguided needle biopsy was performed. Fine-needle aspiration and flow cytometry had negative results for lymphoproliferative disorders or a malignant condition. A tissue culture had negative results. Given the inconclusive diagnosis, the patient underwent a left neck dissection of levels 2A, 2B, and 5A. Intraoperative frozen sections did not show a malignant process. On final analysis, a pathologic examination of the largest lymph node was performed (Figure 1B). Diagnosis D. Castleman disease DiscussionCastleman disease (CD) is an uncommon, lymphoproliferative dis-

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Clinical and pathological characteristics of HIV- and HHV-8–negative Castleman disease

Abstract: Key Points HIV-negative UCD and iMCD are heterogeneous at the clinical, immunophenotypic, and pathologic levels. Complete surgical resection is the primary option of treatment of UCD, while siltuximab is more effective for iMCD than rituximab.

Cited by 134 publications

References 40 publications

Castleman disease in pediatrics: Insights on presentation, treatment, and outcomes from a two‐site retrospective cohort study

Castleman disease in pediatrics: Insights on presentation, treatment, and outcomes from a two‐site retrospective cohort study

Role of Allogeneic Stem Cell Transplant in Chronic Lymphocytic Leukemia and its Implications in Current Era

An Abnormal Neck Mass in a Pediatric Patient

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