Large B cell lymphoma presenting initially in bone marrow, liver and spleen: an aggressive entity associated frequently with haemophagocytic syndrome

Yeh, Yu Min; Chang, Kung Chao; Chen, Ya Ping; Kao, Lin Yuan; Tsai, Huey Pin; Ho, Chung Liang; Wang, Jen Ren; Jones, Dan; Chen, Tsai Yun

doi:10.1111/j.1365-2559.2010.03709.x

Cited by 32 publications

(45 citation statements)

References 34 publications

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“…The following criteria were used for diagnosing lymphoid neoplasia in the BM as PBML: pathologically confirmed BM infiltration with NHL, regardless of peripheral blood involvement; no evidence of lymph‐node involvement, defined as enlargement of any lymph‐node to >1 cm on imaging studies (including thoracic, abdominal, and pelvic computerized tomography [CT] scans); the absence of tumor formation; and exclusion of leukemia/lymphoma cases with primarily BM involvement, including chronic lymphocytic leukemia/small lymphocytic lymphoma, prolymphocytic leukemia, lymphoplasmacytic lymphoma, mantle cell lymphoma, splenic marginal zone lymphoma, hairy‐cell leukemia, Burkitt lymphoma, and acute lymphoblastic leukemia 2, 3, 4…”

Section: Methodsmentioning

confidence: 99%

“…However, several recently reported cases exhibited an uncommon NHL, without lymphadenopathy but only a primary tumor in the BM—primary bone marrow lymphoma (PBML) 1, 2, 3, 4, 5, 6…”

Section: Introductionmentioning

confidence: 99%

“…The current knowledge regarding PBML has mainly been derived from several clinicopathologic reports,1, 2, 3, 4, 5, 6 and most studies of PBML report two diagnostic criteria in common: pathological evidence of BM infiltration with NHL and the absence of any lymph‐node enlargement or tumor formation 1, 2, 3, 4. However, there are two major limitations to our current understanding of this condition.…”

Section: Introductionmentioning

confidence: 99%

“…First, the definitions of PBML used in previous studies varied, partly due to its rarity, heterogeneous histology, and a lack of uniform pathological features 1, 2, 3, 4. Second, PBML often initially manifests as prolonged fever or fever of unknown origin (FUO), which is usually first examined by general practitioners 1, 2, 3, 4, 5, 7, 8, 9, 10. Hence, the diagnosis of PBML is often delayed, leading to a poor prognosis and a median overall survival (OS) of <18 months 1, 2, 3, 4.…”

Section: Introductionmentioning

confidence: 99%

“…Second, PBML often initially manifests as prolonged fever or fever of unknown origin (FUO), which is usually first examined by general practitioners 1, 2, 3, 4, 5, 7, 8, 9, 10. Hence, the diagnosis of PBML is often delayed, leading to a poor prognosis and a median overall survival (OS) of <18 months 1, 2, 3, 4. Therefore, it is essential that hematologists can recognize patients with underlying PBML at an early stage, when they are consulted regarding FUO patients.…”

Section: Introductionmentioning

confidence: 99%

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Primary bone marrow lymphoma: A hematological emergency in adults with fever of unknown origin

et al. 2018

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Primary bone marrow lymphoma (PBML) represents non‐Hodgkin lymphoma (NHL) that primarily arises in the bone marrow (BM) without lymphadenopathy. This condition has various definitions and can be masked by prolonged fever, leading to delayed diagnosis. We aimed to identify clinical features and risk indicators of PBML. We enrolled 269 adults with fever of unknown origin (FUO) who underwent a BM study for potential PBML. Thirty patients were diagnosed with PBML (26 and 4 patients in the training and validation cohort, respectively), and 20 patients (67%) showed initial manifestation of hemophagocytic lymphohistiocytosis (HLH). Among PBML patients in the training cohort, their median overall survival is short (8 days), with pneumonia being the most common direct cause of early mortality, followed by life‐threatening HLH. Despite extremely poor prognoses, some B‐cell PBML patients who survived 30 days after BM studies achieved long‐term survival with rituximab‐based treatment. To assist general practitioners in early PBML diagnosis when approaching adults with naïve FUO, we identified several risk indicators, including elevated serum alkaline‐phosphate levels, lowered serum immunoglobulin‐G levels, cytopenia in ≥2 lineages, and peripheral blood leukoerythroblastosis. Our recently published scoring system, which can predict hematological BM disease in FUO adults, showed excellent ability in recognizing PBML early, with high sensitivity and specificity. We conclude that PBML is a specific “clinical” phenotype of NHL; moreover, we have identified diagnostic clues for early identification of FUO adults with underlying PBML, which should be considered a hematological emergency once suspected in any adult with FUO.

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Section: Methodsmentioning

confidence: 99%

“…However, several recently reported cases exhibited an uncommon NHL, without lymphadenopathy but only a primary tumor in the BM—primary bone marrow lymphoma (PBML) 1, 2, 3, 4, 5, 6…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Primary bone marrow lymphoma: A hematological emergency in adults with fever of unknown origin

et al. 2018

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A Diffuse Medullary Hypercaptation With No Bone Lesion

2020

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A 71-year-old man had a 3-year history of a recurrent seronegative autoimmune constrictive pericarditis and pleural effusions, which were treated with pericardiectomy and prednisone and azathioprine. The pleural fluid was transudative and sterile, and the pericardium contained focal lymphoplasmacytic aggregates. Attempts to taper immunosuppressive drugs failed because of a recrudescence of the inflammatory syndrome and pericardial effusion. The patient was admitted with fever of unknown origin with a surge of the inflammatory syndrome and pancytopenia. He reported night sweats beginning 3 weeks prior associated with fever and unintentional weight loss. No lymphadenopathy was noted.Laboratory evaluations demonstrated a C-reactive protein level of 133 mg/L (reference range, <10 mg/L); ferritin level of 2607 ng/mL (reference, 30-300 ng/mL); lactate dehydrogenase (LDH) level of 289 U/L (reference, 135-214 U/L); pancytopenia with a hemoglobin level of 9.3 g/dL (reference, 13.3-17.7 g/dL), white blood cell count of 2700/μL (reference, 4000-10 000/μL), and platelet count of 99 ×10 3 /μL (reference, 150-350 ×10 3 /μL); and prerenal acute kidney injury (Acute Kidney Injury Network stage 1) with a serum creatinine level of 1.4 mg/dL (reference, 0.7-1.2 mg/dL). Results of a comprehensive serologic workup were negative. Gastroscopy results showed mycotic distal esophagitis.The patient was readmitted 3 weeks later with fever and hypotension without improvement despite broad-spectrum antibiotics and antifungals. Considering the increase in ferritin and LDH levels and pancytopenia, a hematologic disease was suspected. The patient did not report any bone pain. Computed tomography (CT) results showed a splenomegaly without evidence of metastatic disease or lymph node enlargement. Positron emission tomography (PET)-CT imaging (Figure, A and B) and a bone-marrow biopsy (Figure, C and D) were performed. A rapidly evolving sepsislike syndrome led to the patient's death.

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Distribution and clinical features of lymphomas involving skin in Taiwan

Chang

Lee

et al. 2023

The Kaohsiung J of Med Scie

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A wide variety of primary and secondary lymphoma types involves the skin. However, reports with comparisons between both groups are limited in Taiwan. We retrospectively enrolled all cutaneous lymphomas and evaluated their clinicopathologic features. There were 221 cases of lymphoma: 182 (82.3%) primary and 39 (17.7%) secondary. Mycosis fungoides was the most common primary T-cell lymphoma, 92 (41.7%) cases, followed by CD30-positive T-cell lymphoproliferative disorders including lymphomatoid papulosis (n = 33, 14.9%) and cutaneous anaplastic large cell lymphoma (n = 12, 5.4%). The most frequent primary B-cell lymphomas were marginal zone lymphoma (n = 8, 3.6%) and diffuse large B-cell lymphoma (DLBCL), leg type (n = 8, 3.6%). DLBCL including variants was the most common secondary lymphoma involving skin. Most primary lymphomas presented at low-stage (T-cell, 86%; B-cell, 75%), whereas the majority of secondary lymphomas presented at high-stage (T-cell, 94%; B-cell, 100%). Patients with secondary lymphomas had an older mean age, more frequent B symptoms, lower serum albumin and hemoglobin, and a higher frequency of atypical lymphocytes in blood than those with primary lymphomas. In primary lymphomas, older age, lymphoma types, decreased lymphocyte counts and

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Large B cell lymphoma presenting initially in bone marrow, liver and spleen: an aggressive entity associated frequently with haemophagocytic syndrome

Abstract: Although it shares with intravascular LBCL a subtle presentation and an aggressive clinical course, this primary BLS large cell lymphoma variant is distinguished by lacking an intravascular component and having different cytogenetic findings.

Cited by 32 publications

References 34 publications

Primary bone marrow lymphoma: A hematological emergency in adults with fever of unknown origin

Primary bone marrow lymphoma: A hematological emergency in adults with fever of unknown origin

A Diffuse Medullary Hypercaptation With No Bone Lesion

Distribution and clinical features of lymphomas involving skin in Taiwan

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