HIV-negative Primary Bone Marrow Hodgkin Lymphoma Manifesting with a High Fever Associated with Hemophagocytosis as the Initial Symptom: A Case Report and Review of the Previous Literature

Morita, Yasuyoshi; Emoto, Masakatsu; Serizawa, Kentaro; Rai, Shinya; Hirase, Chikara; Kanai, Yoshitaka; Ohyama, Yasuyo; Shiga, Toshihiko; Tanaka, Hirokazu; Miyatake, Jun-ichi; Tatsumi, Yoichi; Ashida, Takashi; Kimura, Masatomo; Ito, Masafumi; Matsumura, Itaru

doi:10.2169/internalmedicine.54.3770

Cited by 6 publications

(6 citation statements)

References 10 publications

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“…Unfortunately, more than half of these patients died in the short term, and disease progression was the dominant cause of death. Morita et al21 reported that HIV-negative cases tended to progress rapidly and resulted in worse outcomes, which is consistent with our analysis. Regarding T-cell lymphoma, we first used the gemcitabine, cisplatin, prednisone, and thalidomide regimen in our patients because this regimen was proven to be more efficient than CHOP for the treatment of PTCL in a prospective, randomized, controlled, and open-label clinical trial;22 however, the results were not satisfactory and the results of the CHOP regimen were also not satisfactory.…”

Section: Discussionsupporting

confidence: 93%

<p>Clinical features and prognostic factors of primary bone marrow lymphoma</p>

Wang¹,

Chang²,

Wu³

et al. 2019

CMAR

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Background Primary bone marrow lymphoma (PBML) is a very uncommon neoplasm originally arising in the bone marrow system, and the most common pathological type is diffuse large B-cell lymphoma. Patients and methods To describe the clinical characteristics of PBML and evaluate the risk factors related to prognosis, we recruited and studied 66 patients from our center and the current published literature. Various symptoms are present at the onset of PBML, the most important of which is cytopenia, followed by fever. Forty-seven of these patients were included in our analysis. Results Univariate analysis suggested that B symptoms ( P =0.024), a low serum platelet level (<75×10 9 /L; P =0.032), an elevated serum LDH level ( P =0.039), and not achieving a complete response (CR) following initial therapy ( P =0.007) are associated with worse outcomes. Multivariate analysis showed that only a low serum platelet level (<75×10 9 /L), B symptoms, and not achieving a CR following initial therapy are independent factors for prognosis. In addition, intensive regimens appear to be beneficial for prognosis. Conclusion PBML is a lymphoma with special clinical features, and its recognition is important for establishing a definitive prognosis model and searching for appropriate therapy.

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Section: Discussionsupporting

confidence: 93%

<p>Clinical features and prognostic factors of primary bone marrow lymphoma</p>

Wang¹,

Chang²,

Wu³

et al. 2019

CMAR

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“…As no malignant cells were found outside the bone marrow, it is possible that this represented primary bone marrow Hodgkin lymphoma. While this is quite rare and typically associated with HIV infection, for which our patient tested negative, several previous cases have been described of HIV-negative primary bone marrow Hodgkin lymphoma [ 35 , 36 ]. Alternatively, it is possible that the tonsillar biopsy was a false negative and that this represented stage 4 Hodgkin lymphoma.…”

Section: Discussionmentioning

confidence: 70%

An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin Lymphoma

Komisarof

Mcgann

Huston

et al. 2021

Case Reports in Hematology

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Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome of immune system dysregulation characterized by the phagocytosis of various cells by histiocytes in the bone marrow. HLH can present in one of the two ways: primary HLH, which is caused by mutations in genes essential to T and NK-cell function, and secondary HLH, typically caused by Epstein–Barr virus (EBV) infection or malignancy. Because of the rapid progression and high mortality of this disease, prompt diagnosis is essential to good outcomes. Here, we report the 2-month clinical course of a patient who presented with altered mental status and recurrent fever of unknown origin. Initially, he did not meet diagnostic criteria for HLH and had a negative bone marrow biopsy; however, he eventually progressed to full-blown HLH secondary to occult Hodgkin lymphoma. This case is unusual for the slow and smoldering course of the patient’s disease and highlights the importance of aggressively searching for potential malignancies to ensure the initiation of definitive therapy as soon as possible.

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“…Therefore, bone marrow biopsy continues to be the gold standard to confirm the involvement of the bone marrow in HL patients with PET/CT employed for follow-up. So far, there are only seven cases of PBMHL reported (Table 1) [10][11][12][13][14][15]. As outlined above, the current first-line combination chemotherapy for HL is ABVD.…”

Section: Discussionmentioning

confidence: 99%

Immunocompetent Patient With Primary Bone Marrow Hodgkin Lymphoma

Dhaliwal

Eller

2022

J Med Cases

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Hodgkin lymphoma (HL) is a hematologic malignancy that comprises about 10% of all lymphomas with the most common type being classical HL (cHL). The typical clinical presentation of cHL involves multiple region lymphadenopathy and a chest mass found on imaging. However, not all patients present with the typical symptomology of cHL which poses a diagnostic challenge. Extranodal HL, especially primary bone marrow HL (PBMHL), has been described in immunocompromised patients with human immunodeficiency virus (HIV). In this case report, we present a PBMHL case in an immunocompetent patient with no HIV exposure. We discuss a 51-year-old immunocompetent female who presented with 2 -3 months of fever, confusion, generalized myalgias, and fatigue. She had no lymphadenopathy on physical exam. On further testing, the patient's blood work demonstrated cytopenia and imaging confirmed no lymphadenopathy. Eventually, a bone marrow evaluation established her diagnosis of PBMHL. The patient expired after receiving one cycle of a modified chemotherapy regimen. This case illustrates that HL can be associated with an atypical clinical presentation which may delay diagnosis and treatment. PBMHL can occur in the normal population who is not immunocompromised nor HIV positive. In this situation, the best diagnostic approach is a thorough medical history, physical exam, and bone marrow aspiration and biopsy. Presence of constitutional symptoms without any lymphadenopathy or chest mass should raise the concern for possible atypical HL such as PBMHL. Accurate and timely identification of PBMHL allows for timely initiation of appropriate therapy. While cHL is responsive to chemotherapy, further research is required to improve the therapy for PBMHL.

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HIV-negative Primary Bone Marrow Hodgkin Lymphoma Manifesting with a High Fever Associated with Hemophagocytosis as the Initial Symptom: A Case Report and Review of the Previous Literature

Cited by 6 publications

References 10 publications

<p>Clinical features and prognostic factors of primary bone marrow lymphoma</p>

<p>Clinical features and prognostic factors of primary bone marrow lymphoma</p>

An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin Lymphoma

Immunocompetent Patient With Primary Bone Marrow Hodgkin Lymphoma

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