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ORIGINAL ARTICLE
Year : 2016  |  Volume : 12  |  Issue : 2  |  Page : 763-764

An analysis of autopsy cases of non-Hodgkin lymphoma-with special reference to those masquerading as acute febrile illness


Department of Pathology, Lokmanya Tilak Municipal Medical College, Mumbai, Maharashtra, India

Date of Web Publication25-Jul-2016

Correspondence Address:
Sonali Rajesh Saraf
Department of Pathology, C-404, Siddhivinayak Annexe, SJ Road, Lower Parel West, Mumbai - 400 013, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.158028

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 > Abstract 


Context: As in any medical disorder, in non-Hodgkin lymphomas (NHLs) also, precise analysis of causes of death is needed to focus research efforts and improve morbidity and mortality.
Aims: The aim of this study was to review the clinical presentation, mode of death and the immunophenotype of the autopsy cases of NHL.
Settings and Design: Autopsy cases wherein NHL was diagnosed, were selected for study.
Subjects and Methods: Autopsy cases which were diagnosed as NHL, either antemortem or after autopsy during a 7 years period at a tertiary care referral centre were studied and reviewed.
Statistical Analysis Used: Descriptive statistical analysis used.
Results: The autopsy findings seen in eight cases of NHL were reviewed. Except one, all cases were above 40 years age. Infective etiology (62%) followed by cardiorespiratory failure (38%) was the cause of death observed in these patients. In three cases, the antemortem diagnosis of NHL was missed as the patients were being treated for acute febrile illness and were referred late to the Tertiary Care Centre. One of these was a case of extra-nodal primary splenic lymphoma.
Conclusion: As NHL present with nonspecific symptoms, these tumours may not be detected in early stages and hence may not be treated appropriately. These patients have weakened immunity and hence are prone to infection and sepsis which can be a major cause of mortality. This autopsy study experience has shown that NHL can masquerade as acute febrile illness which if not detected early and treated adequately can turn fatal.

Keywords: Acute febrile illness, autopsy, non-Hodgkin lymphoma


How to cite this article:
Saraf SR, Naphade NS, Kalgutkar AD. An analysis of autopsy cases of non-Hodgkin lymphoma-with special reference to those masquerading as acute febrile illness. J Can Res Ther 2016;12:763-4

How to cite this URL:
Saraf SR, Naphade NS, Kalgutkar AD. An analysis of autopsy cases of non-Hodgkin lymphoma-with special reference to those masquerading as acute febrile illness. J Can Res Ther [serial online] 2016 [cited 2019 Dec 9];12:763-4. Available from: http://www.cancerjournal.net/text.asp?2016/12/2/763/158028




 > Introduction Top


Precise analysis of causes of death is needed to focus research efforts and improve morbidity and mortality. The non-Hodgkin lymphomas (NHLs) represent a heterogeneous group of neoplastic diseases with differences in clinical presentation, histologic appearance, clinical course and response to therapy.[1] They are prone to infection and sepsis and can thus mislead the clinicians to diagnose as acute febrile illness especially when presenting with symptoms of fever with thrombocytopenia. The aim of this study was to stress the importance of early detection and diagnosis of NHLs which can present as acute febrile illness.


 > Subjects and Methods Top


Autopsy cases which were diagnosed as NHL, either antemortem or after autopsy during a 7 years period at a Tertiary Care Referral Centre were studied. Case histories were retrieved by going through indoor case papers from medical records office. The autopsy histopathology slides of cases which had died of hemato-lymphoid disorder were reviewed. Cases diagnosed as lymphomas were subtyped with the help of immunohistochemistry.


 > Results Top


Out of 4378 autopsies performed in 7 years period, the deaths due to hemato-lymphoid disorders accounted for 48 cases, its incidence being 1.09%. NHL was diagnosed in eight cases (16.6%). All cases were above 40 years age except one who was 21 years of age. Most of the patients presented with fever (87.5%). Others had abdominal pain (75%), dyspnea, cough (37.5%), vomiting (25%), altered sensorium and bone pain (12.5%). In spite of the availability of modern ancillary modes of investigations like computed tomography (CT) and ultrasonography (USG), three patients were misdiagnosed clinically as acute febrile illness probably because of the clinical presentation of fever and mild thrombocytopenia and were being treated with antibiotics. The hospital stay of these cases was variable from 1 to 3 days. Generalised lymphadenopathy was seen in one of these three cases while peripancreatic and mesenteric lymphadenopathy was seen in second while the third case showed primary splenic involvement. Of these, two cases were labelled as T-cell lymphomas namely anaplastic large T-cell lymphoma and angioimmunoblastic T-cell lymphoma while one case was diagnosed as primary diffuse large B-cell lymphoma (DLBCL) of the spleen. T-cell lymphomas are known to have poor prognosis and detection rate.

Non-Hodgkin lymphoma is generally associated with peripheral lymphadenopathy, hepatosplenomegaly or both. This was seen in 62.5%, 37.5% and 12.5% of cases respectively.

The lymph nodes below diaphragm were more commonly involved (in 6/8 cases).

Infective etiology (62%) followed by cardiorespiratory failure (38%) was the cause of death observed in these patients.

Two cases of extra-nodal lymphomas were observed. Of these one had Burkitt lymphoma with primary mandibular involvement and the other had primary splenic DLBCL.

Immunohistochemistry was done in seven cases showed a predominance of B-cell lymphoma (four cases). T-cell lymphoma with CD3 positivity was seen in two cases. In one case transformation of follicular lymphoma into more aggressive DLBCL was noted, however it was negative for Bcl2.


 > Discussion Top


Overall, autopsy provides important information for general clinical practice and also in patients with complex hematological diseases.[2] Hematological disorders constitute a special group of diseases due to their low incidence, high mortality, complex diagnostic tests that are required for their care and for the need of aggressive therapeutic methods. Patients with varied clinical presentation are at increased risk to suffer from misdiagnosis leading to discrepancy in clinical diagnosis.[2]

The incidence of autopsies of hemato-lymphoid disorders was less in our institution as compared to the studies of Xavier et al.[2] and Tavora et al.[3] The incidence of diagnosis that was missed ante mortem in the present study was comparable with the study by Xavier et al.[2] but was higher than that of Tavora et al.[3]

Non-Hodgkin lymphoma clinically presents with nonspecific symptoms such as weight loss and fatigue, followed by acute abdomen pain. NHL was clinically not suspected in three cases because the patients presented in critical conditions with symptoms similar to acute febrile illness. Cases of lymphoma with clinical course mimicking fever of unknown origin and sepsis have been described in literature.[4] An unusual presentation of anaplastic large cell lymphoma with clinical course mimicking fever of unknown origin and sepsis has been described in autopsy study of five cases by Mosunjac et al.[5]

The main problem in such patients is to detect these tumors as early as possible with the help of USG/CT because prognosis is related to tumor stage. If we could detect these tumors earlier, prognosis would be better. As these cases present with nonspecific symptoms, these tumours are not detected in early stages and hence not treated appropriately.

Infections and cardiorespiratory failure were the leading cause of death which was also noted by other studies.[1],[4] Predisposing factors for infection included the underlying disease, (i.e., lymphomatous infiltration of organ systems) and granulocytopenia secondary to combination chemotherapy. Respiratory failure resulted due to secondary lymphomatous infiltration of the lung.

These patients have weakened immunity and hence are prone to infection and sepsis which can be a major cause of mortality. This autopsy study experience has shown that NHL can masquerade as acute febrile illness which if not detected early and treated adequately can turn fatal.

 
 > References Top

1.
Hyman CB, Bogle JM, Brubaker CA, Williams K, Hammond D. Central nervous system involvement by leukemia in children. I. Relationship to systemic leukemia and description of clinical and laboratory manifestations. Blood 1965;25:1-12.  Back to cited text no. 1
    
2.
Xavier AC, Siqueira SA, Costa LJ, Mauad T, Nascimento Saldiva PH. Missed diagnosis in hematological patients-an autopsy study. Virchows Arch 2005;446:225-31.  Back to cited text no. 2
    
3.
Tavora F, Crowder CD, Sun CC, Burke AP. Discrepancies between clinical and autopsy diagnoses: A comparison of university, community, and private autopsy practices. Am J Clin Pathol 2008;129:102-9.  Back to cited text no. 3
    
4.
Lantz B, Adolfsson J, Lagerlöf B, Reizenstein P. Causes of death in leukemia and lymphoma with modern treatment. Acta Haematol 1980;63:61-7.  Back to cited text no. 4
    
5.
Mosunjac MB, Sundstrom JB, Mosunjac MI. Unusual presentation of anaplastic large cell lymphoma with clinical course mimicking fever of unknown origin and sepsis: Autopsy study of five cases. Croat Med J 2008;49:660-8.  Back to cited text no. 5
    




 

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