A Challenging Case of A Myeloid Sarcoma Misdiagnosed as High Grade B-Cell Lymphoma

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S97-S97
Author(s):  
A Herrmann ◽  
B Mai ◽  
S Elzamly ◽  
A Wahed ◽  
A Nguyen ◽  
...  
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Myeloid Sarcoma ◽  
Proliferation Index ◽  
High Grade ◽  
Cell Markers ◽  
Thoracolumbar Region ◽  
The Right

Abstract Introduction/Objective A 46-year-old female presented with severe back pain associated with progressive bilateral lower extremity weakness and paresthesia, urinary retention, and constipation. Computed tomography revealed a retroperitoneal mass encasing the right psoas muscle, obstructing the right kidney, and extending to the thoracolumbar region resulting in severe spinal compression. An epidural tumor resection was subsequently performed at an outside hospital. Methods Histological sections showed sheets of blastoid neoplastic cells with intermediate to large nuclei, irregular membranes, fine chromatin, and prominent nucleoli. Immunohistochemical stains showed that these cells were positive for CD43, CD79a (weak, focal), BCL2, C-MYC, and PAX5 (weak, focal) and negative for CD10, CD20, CD30, ALK1, BCL6, MUM1, and Tdt. The Ki-67 proliferation index was 75-80%. With this immunophenotype, this patient was diagnosed with a high grade B-cell lymphoma and transferred to our institution for further work-up. On review of the slides, further immunohistochemical testing was requested which revealed positivity for CD117 and myeloperoxidase (MPO). Results The overall morphological and immunophenotypical features are most compatible with myeloid sarcoma (MS) with aberrant expression of B-cell markers and this patient’s diagnosis was amended. Interestingly, the patient’s bone marrow examination only showed 2% myeloblasts with left shifted granulocytosis and concurrent fluorescence in situ hybridization (FISH) studies were negative. Conclusion A literature review showed that 40-50% of MS are misdiagnosed as lymphoma. MS can frequently stain with B-cell or T-cell markers, as seen in this case, which makes it challenging for an accurate diagnosis and sub- classification. In addition, our case is interesting in that there was only extramedullary presentation without bone marrow involvement. Typically, MS develops after the diagnosis of acute myeloid leukemia (AML) with an incidence of 3–5% after AML. It can also manifest de novo in healthy patients, who then go on to develop AML months to years later. Therefore, this patient will require close follow-up.

Concurrent Chromosomal Alterations at 3q27, 8q24 and 18q21 in An Atypical Form of Burkitt’s Lymphoma

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5268-5268
Author(s):  
Majdi SM Hamarshi ◽  
Maha abu Kishk ◽  
Mahmoud Mahafzah ◽  
Jami Walloch
Keyword(s):  
Bone Marrow ◽  
Follicular Lymphoma ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Burkitt’S Lymphoma ◽  
Burkitt's Lymphoma ◽  
High Grade ◽  
Surface Immunoglobulin ◽  
Cell Neoplasm

Abstract Introduction: Chromosomal translocations are common in non-Hodgkin’s lymphomas (NHL), most frequently involving the genes bcl-2 in the t(14;18) of follicular lymphoma (FL), c-myc in the t(8;14) of Burkitt’s lymphoma (BL) and bcl-6 in the t(3;14) of follicular or diffuse large B-cell (DLBC) lymphoma. We report the clinical features, pathology and genetic findings in an exceedingly rare case of Burkitt’s lymphoma that showed concurrent involvement of these three chromosomal loci. Case Report: This is a 65 year old Caucasian female who presented with a rapidly growing right supraclavicular lymph node over a few weeks. FNA biopsy showed typical morphology of Burkitt’s lymphoma. Similar morphologic features were found on the bone marrow biopsy. There was widespread disease with no CNS involvement. Flow cytometry from peripheral blood and immunohistochemistry on the cellblock showed B-cell phenotype positive for CD 10, CD19, CD20 (negative CD20 by immunohistochemistry), HLA-DR, cytoplasmic CD79a, and negative for CD34 and TdT. The interesting finding was the lack expression of surface or cytoplasmic immunoglobulin and expression of weak Bcl-6. Almost 90–95% expressed Ki67. The cytogenetic analysis reportedly demonstrated a complex karyotype t(3;8;14), and t(14;18) involving c-myc (8q24), bcl-2 (18q21), and bcl-6 (3q27). After 7 cycles of hyper CVAD-R she had bone marrow biopsy which showed residual disease. She also had a biopsy confirmed relapse as left arm nodule and left leg nodular infiltrate at 8 and 12 months form the diagnosis, respectively. Discussion: This is a complex case of high grade B-cell lymphoma with morphology suggestive of Burkitt’s lymphoma. However the classification was challenging by the lack of surface immunoglobulin expression that might be expected in mature B-cell neoplasm “DLBCL, FL”, and the lack of TdT and CD34 that might be expected in precursor B-cell neoplasm “BL”. The diagnosis was highly dependent on the cytogenetic findings, which was significant for the presence of t(8;14) albeit in a three way translocation t(3;8;14), and t(14;18) involving c-myc (8q24), bcl-2 (18q21), and bcl-6 (3q27). The lymphoma was therefore described as “Burkitt’s transformation”. This is a rare translocation pattern, but has been described in follicular lymphoma, grade 3; diffuse large cell lymphoma; and Burkitt’s lymphoma. Conclusion: BL might lack surface immunoglobulin expression making the diagnosis of high grade B-cell lymphoma challenging if based on the morphology and immunophenotyping alone. The cytogentetic findings better delineate sub-types of lymphoma. Molecular evidence of multiple oncogene deregulations, especially when involving the c-myc gene, appears to be associated with a dire clinical outcome.

High-Grade Diffuse Large B-Cell Lymphoma in Elderly Patients: Russian Multicenter Trial Results in R-EPOCH/R-HMA Protocol

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5386-5386
Author(s):  
Olga A. Gavrilina ◽  
Eugene E. Zvonkov ◽  
Elena N. Parovichnikova ◽  
Nelly G. Gabeeva ◽  
Vera V. Troitskaya ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Elderly Patients ◽  
B Cell ◽  
Older Patients ◽  
Cell Lymphoma ◽  
Bone Marrow Involvement ◽  
B Cell Lymphoma ◽  
High Grade ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Abstract Background: The number of elderly patients with diffuse large B-cell lymphoma (DLBCL) in our aging society continues to rise. Median of age for patients with diffuse large B-cell lymphoma (DLBCL) is 60. Approximately 50% of older patients with DLBCL are defined as high-grade by IPI and these forms are characterized by aggressive course and poor response to standard chemotherapy (CT). Intensive protocols cannot be performed due to their toxicity for older patients with comorbidity. Addition of R-HMA to R-DA-EPOCH favourably changes the outcome in patients with untreated high-grade diffuse large B-cell lymphoma and didn't have higher toxicity [ASH 2015 # 2708]. Aim: To evaluate the efficacy and toxicity of R-EPOCH/R-HMA protocol in older patients with untreated high-grade diffuse large B-cell lymphoma. Patients and Methods: 19 untreated older DLBCL patients from 4 centers were enrolled in a prospective study between August 2013 - July 2016; stage II-IV; ECOG 0-3; median age 66 years (60-78); age ≥70y/60<70y 21%/79%; M/F 52%/48%; IPI: 52% high-intermediate and 48% high risk; 26% with bone marrow involvement. Severe comorbidity was diagnosed in 8 (42%) patients (coronary heart disease, hypertonic disease, chronic obstructive pulmonary disease and arrhythmia). All patients underwent 4-6 courses (2-3 cycles) of chemotherapy: R-EPOCH (standard dose and scheme), R-HMA (R 375 mg/m2 d1, MTX 500 mg/m2 24 hours d 2, AraC 1000 mg/m2 q 12 hrs d 3-4). In 3 cases of DLBCL with bone marrow involvement BEAM conditioning and autologous stem cell transplantation were applied. Results: The median follow-up is 18 months (3-37). There was no mortality associated with toxicity. The main non-hematological toxicities of R-HMA were infections (mucositis, pneumonia, sepsis, enteropathy) grades 1-2 and 3-4 in 90% and 10%, respectively. Hematological toxicity grade 4 for less than 4 days we observed only after courses R-HMA. Complete remission (CR) was achieved in 18 (100%) patients and 1 patient in the treatment now. There are four failures in patients older than 60 years: three relapses (after 6 and two after 14 month CR) and one death after 7 month CR by reasons not related with DLBCL. With a median follow 18 months overall and event-free survival of 19 older patients constituted 93,8% and 75,9%, respectively (Fig.1). There is no difference in older patients according to stage, IPI, LDH level, ECOG status for OS and EFS. So the combination of R-EPOCH/R-HMA may be considered as optimal intensive approach in older patients. Conclusions: TheR-EPOCH/R-HMA protocol demonstrated acceptable toxicity and high efficacy in older patients with high-grade DLBCL. Figure 1 Overall (A) and Event-free (B) survival in elderly patients with DLBCL. Figure 1. Overall (A) and Event-free (B) survival in elderly patients with DLBCL. Disclosures No relevant conflicts of interest to declare.

scholarly journals Role of Immunohistochemistry in Staging Diffuse Large B-cell Lymphoma (DLBCL)

2008 ◽  
Vol 56 (10) ◽  
pp. 893-900 ◽  
Author(s):  
Dipti Talaulikar ◽  
Jane Esther Dahlstrom ◽  
Bruce Shadbolt ◽  
Amy Broomfield ◽  
Anne McDonald
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Cox Regression Analysis ◽  
Cell Markers ◽  
Large B Cell Lymphoma ◽  
Lymphomatous Involvement ◽  
The Impact ◽  
Large B Cell

The use of immunohistochemistry (IHC) in staging bone marrow in non-Hodgkin's lymphoma (NHL) is largely limited to ambiguous cases, particularly those with lymphoid aggregates. Its role in routine clinical practice remains unestablished. This study aimed to determine whether the routine use of IHC in diffuse large B-cell lymphoma (DLBCL) would improve the detection of lymphomatous involvement in the bone marrow. It also sought to determine the impact of IHC on predicting survival compared with routine histological diagnosis using hematoxylin and eosin (H&E), Giemsa, and reticulin staining. The bone marrow trephines of 156 histologically proven DLBCL cases were assessed on routine histology, and IHC using two T-cell markers (CD45RO and CD3), two B-cell markers (CD20 and CD79a), and κ and λ light chains. IHC detected lymphomatous involvement on an additional 11% cases compared with histology alone. Although both routine histology and IHC were good predictors of survival, IHC was better at predicting survival on stepwise multivariate Cox regression analysis. IHC performed routinely on bone marrow trephines has the ability to improve detection of occult lymphoma in experienced hands. Furthermore, it is a better predictor of survival compared with routine histological examination alone.

Transformation of Follicular Lymphoma to a High Grade B-Cell Lymphoma with MYC and BCL2 Translocations and Overlapping Features of Burkitt Lymphoma and Acute Lymphoblastic Leukemia

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5421-5421
Author(s):  
David M Aboulafia ◽  
Alina Bischin ◽  
Russell K. Dorer
Keyword(s):  
Bone Marrow ◽  
Ct Scan ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
High Grade ◽  
Pet Ct ◽  
Atypical Cells ◽  
Small Focus ◽  
Fdg Pet Ct

Abstract Most commonly, histologic transformation (HT) from follicular lymphoma (FL) manifests as a diffuse large B cell lymphoma, not otherwise specified (DLBCL, NOS). Less frequently, HT may result in a high-grade B cell lymphoma with MYC and BCL2 and/or BCL6 gene rearrangements, also known as "double-hit" or "triple-hit" lymphomas. In the 2016 revision of the WHO classification of lymphoid neoplasms, the category B-cell lymphoma, unclassifiable was eliminated due to its vague criteria and limiting diagnostic benefit. Instead, the WHO introduced the high-grade B-cell lymphoma (HGBL) category, characterized by MYC and BCL2 and/or BCL6 rearrangements. Cases that present as an intermediate phenotype of DLBCL and Burkitt lymphoma (BL) will fall within this HGBL category. Very rarely, HT results in both the intermediate DLBCL and BL phenotype, and exhibits lymphoblastic features, in which case the WHO recommends that this morphological appearance should be noted. In comparison to de-novo patients with DLBCL, NOS, those with MYC and BCL2 and/or BCL6 gene rearrangements have a worse prognosis. A 63-year-old female presented with left neck adenopathy. Lab assessment including complete blood count (CBC), complete metabolic panel (CMR), serum lactate dehydrogenase (LDH) and B-2 microglobulin were all normal. A whole body computerized tomography (CT) scan revealed diffuse adenopathy above and below the diaphragm. An excisional node biopsy showed grade 3A nodular FL. The Ki-67 labeling index was 40-50%. A bone marrow biopsy showed a small focus of para-trabecular CD20+ lymphoid aggregates. She received 6 cycles of bendamustine (90 mg/m2 days +1 and + 2) and rituximab (375 mg/m2 on day+2) with each cycle delivered every 4 weeks. A follow up CT scan at completion of therapy showed a partial response with resolution of axillary adenopathy and a dramatic shrinkage of the large retroperitoneal nodes. 18 months later she had crampy abdominal pain in the absence of B symptoms. Positron emission tomography with 2-deoxy-2-[fluorine-18] fluoro- D-glucose integrated with CT (18F-FDG PET/CT) scan showed widespread adenopathy, diffuse splenic involvement and substantial marrow involvement. Biopsy of a 2.4 cm right axillary node (SUVmax of 16.1) showed involvement by grade 3A FL with a predominant nodular pattern of growth. A bone marrow biopsy once again showed only a small focus of FL. She received idelalisib (150 mg twice daily), and rituximab (375 mg/m2, monthly) beginning May, 2015. After 4 cycles, a repeat CT scan showed a complete radiographic response. Idelalisib was subsequently held while she received corticosteroids for immune-mediated colitis. A month later she restarted idelalisib with a 50% dose reduction. Two weeks later she returned to clinic complaining of bilateral hip and low lumbar discomfort but no B symptoms. A restaging 18F-FDG PET/CT in January of 2016 showed dramatic marrow uptake. A bone marrow aspirate showed sheets of tumor cells representing a spectrum from intermediate sized cells with lymphoblastic features to very large atypical cells with multiple nucleoli. Two distinct histologies were present; one remained consistent with the patient's known FL with a predominant nodular pattern and the other consistent with HT (The large atypical cells expressed PAX5, CD10, BCL2, and cMYC, and were negative for CD20, MPO, CD34, CD30, and BCL6). Focal areas showed faint, heterogeneous expression of terminal deoxynucleotidyl transferase (TdT) best seen on the clot section. Ki67 proliferation index was high (4+/4). FISH analysis showed two populations with MYC amplification and/or rearrangement, and no evidence of BCL6 rearrangement; a karyotype analysis showed a complex abnormal female karyotype with t (14; 18) and multiple structural and numerical abnormalities. She started dose-adjusted rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (DA-R-EPOCH) with concomitant prophylactic intrathecal methotrexate and cytarabine. She had but a short lived response before dying in hospice from progressive lymphoma. Whether idelalisib could provide a microenvironment for selection of more aggressive clones needs to be addressed. Our patient's clinical course is confounded by the incorporation of idelalisib while being further complicated by the complexity of HT and the mechanisms in which first-line chemotherapy regimens impact DHL. Disclosures No relevant conflicts of interest to declare.

Blastoid high-grade B-cell lymphoma initially presenting in bone marrow: a diagnostic challenge

2021 ◽  
Author(s):  
Mahsa Khanlari ◽  
L. Jeffrey Medeiros ◽  
Pei Lin ◽  
Jie Xu ◽  
M. James You ◽  
...  
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
High Grade ◽  
Diagnostic Challenge

A review of clinicopathologic characteristics, therapy, and outcomes of 22 patients with aggressive B-cell lymphoma.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e21123-e21123
Author(s):  
Michael Jaglal ◽  
Deniz Peker ◽  
Celeste M. Bello ◽  
Bijal D. Shah ◽  
Lubomir Sokol ◽  
...  
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Proliferation Index ◽  
High Grade ◽  
Female Ratio ◽  
Cns Disease ◽  
Entire Cohort ◽  
Genetic Abnormalities ◽  
Worse Prognosis

e21123 Background: B cell lymphoma, unclassifiable, with features intermediate between diffuse large B cell lymphoma (DLBCL) and Burkitt lymphoma (BL) or Burkitt like lymphoma is a high grade lymphoma that is associated with a high proliferation index and a complex karyotype mostly involving MYC and BCL-2 genes, so called “double hit” as well as BCL-6 or CYCLIND1 genes, so called “triple hit”. This high grade lymphoma shows an aggressive behavior for which the most appropriate therapeutic approach is yet to be established. Methods: This was a single center retrospective review of pts with a confirmed diagnosis of B cell lymphoma, unclassifiable, with features intermediate between DLBCL and BL from 2006 to 2011. The definitive diagnosis in all cases was based on morphological and phenotypic features, and genetic abnormalities involving MYC, BCL-2, BCL-6, and or CYCLIND1. Clinicopathological data was extracted including age, gender, primary disease location, phenotypic subtype, genetic abnormalities by FISH, IPI scores, treatment regimens and survival data . Descriptive statistical analyses were utilized. Kaplan-Meier method was used to estimate OS and log rank test was used to compare the groups. All data were analyzed using SPSS version 20.0 statistical software. Results: 22 pts with &quot;Double or Triple Hit&quot; lymphomas were identified between 2006 and 2011. The age range at diagnosis was 33-87 years with median age of 66. 16 of 22 pts (73%) were ≥ 60 years old. Male to female ratio was 1.75 :1 (14:8). The median ECOG PS was 1. No pts had HIV. A majority of pts presented with extranodal disease 17 with only 5 pts presenting with nodal disease. The median survival of the entire cohort was 9 months with ranges 32 to 2 months. 16 of 22 pts (73%) are currently alive. In the cohort of pts that are currently alive, 10 was treated with RCHOP and 6 with RHyperCVAD. Pts who presented with CNS disease had a worse prognosis when compared to the entire cohort with a median overall survival of 6.5 months (p=0.016). Conclusions: RCHOP was the most utilized treatment regimen in the cohort of  alive patients. CNS disease is associated with worse prognosis in patients with &quot;Double or Triple Hit&quot; lymphomas.

scholarly journals Aggressive Diffuse Intermediate Size B-Cell Lymphoma With P53 Mutation Presented as Primary Bone Marrow Lymphoma

2020 ◽  
Vol 8 ◽  
pp. 232470962098276
Author(s):  
Pei Ting Chen ◽  
Karan Jorsan ◽  
Boris Avezbakiyev ◽  
Cheema Akhtar ◽  
Jen Chin Wang
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
P53 Mutation ◽  
Rapid Progression ◽  
Low Grade ◽  
High Grade ◽  
Primary Bone ◽  
Primary Bone Marrow

Primary bone marrow lymphoma (PBML) is a disease entity in which lymphoma primarily originates in the bone marrow without signs of involvement of lymph nodes, spleen, liver, or any other organs, and excludes leukemia/lymphoma. PBML has been a rare presentation of malignant lymphoma, and most of the cases have a poor prognosis and require rapid diagnoses and treatments. Among all PBMLs, diffuse large B-cell lymphoma (DLBCL) is the most common pathological subtype. Over 25 years and from 7 institutions, the International Extranodal Lymphoma Study Group retrospectively collected PBML cases and, in 2012, published these 21 cases, including 19 cases of B-cell lymphoma and 2 cases of peripheral T-cell lymphoma. Among the B-cell types, DLBCL accounted for 79% and follicular lymphoma (FL) for 21%. DLBCLs were characterized by the existence of large cells. In this article, we present a rare case of high-grade aggressive type with P53 mutation, intermediate-sized B-cell lymphoma, excluded FL by the absence of FL lymphoma markers, presented as PBML. Our patient had rapid progression and succumbed to the disease shortly after diagnosis. Upon literature review, 62 B-cell lymphoma cases were identified that presented as PBML (51 high-grade and 11 low-grade)—mostly case reports. Among these, only one case was reported as intermediate-sized DLBCL-like lymphoma but not with aggressive features. Our case represents the first case of aggressive intermediate-sized lymphoma, not a FL, with P53 mutation, highly elevated lactate dehydrogenase, and Ki-67 presented as PBML. Such a profile would need to be quickly recognized and aggressive treatment applied, such as CART (chimeric antigen receptor T-cells) therapy or DA-EPOCH-R (dose-adjusted EPOCH [etoposide-prednisone-oncovin-cyclophosphamide-hydroxydaunorubicin] and rituximab) with or without venetoclax.

scholarly journals Presentation of aggressive high-grade B cell lymphoma as venous thromboembolism

2017 ◽  
Vol 5 (9) ◽  
pp. 4175
Author(s):  
Shahnila Ali ◽  
Taarif Hussain
Keyword(s):  
Bone Marrow ◽  
Lymph Node ◽  
Venous Thromboembolism ◽  
B Cell ◽  
Bone Marrow Biopsy ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Urgent Care ◽  
High Grade ◽  
Groin Swelling

Patient with no known past medical history who came to United States few years back and no established PCP noticed right groin swelling and discomfort for 3 days. In addition to right groin swelling and discomfort, she also noticed fatigue especially at work. Due to progressive symptomatology, she seeked help at urgent care. On physical examination performed by urgent care doctor, patient found to have inguinal lymphadenopathy, which led to general surgery referral and subsequent lymph node biopsy a day afterwards. Curious surgeon followed pathology report, which turned out to be Burkitt’s lymphoma. Subsequently, surgeon referred for survey which included CT scans abdomen and pelvis with contrast. Radiologist reading imaging called patent to go to ED STAT as she found massive B/L pulmonary embolism in main pulmonary arteries incidently. Other findings included lymphadenopathy in the right inguinal, right pelvic and peri aortic locations. Mildly enlarged left pelvic lymph node. No lymphadenopathy in the chest. Patient was initiated immediately on heparin infusion on presentation to ED. Oncology consulted and ordered additional studies including ECHO. Patient received bone marrow biopsy and lumbar puncture as well. She was initiated on standard chemotherapy R-EPOCH regimen. Patient also received allopurinol entecavir and bactrim Subsequently, bone marrow biopsy results showed high grade B cell Lymphoma. Staging bone marrow negative. LP showed normal protein, borderline elevation of glucose, flow cytometry and cytology negative for involvement. C-myc translocation, phenotype of Burkitt’s but with lower Ki67. Sites of disease included massive right inguinal adenopathy, peri aortic and left pelvic adenopathy.Patient was followed during the hospital course and had excellent recovery. Symptomatically, she no longer felt fatigued and leg swelling / groin mass significantly improved at time of discharge. She was discharged in stable condition with outpatient follow up with oncology.Aggressive High Grade B cell Lymphoma may present with venous thromboembolism and due to aggressive nature of lymphoma, venous thromboembolism can pose/present with significant clot burden before being diagnosedLymphoma should be considered as a causative disease in a patient even adult with venous thromboembolismAggressive evaluation and prompt treatment is needed for good results and recovery

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