SESSION TYPE: Miscellaneous Global Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Sickle cell disease (HbS) is an inherited disorder due to homozygosity for the abnormal hemoglobin S. Although it is rare in Caucasians , it is a prevalent disease in Saudi Arabia.(1) Vaso-occlusive phenomena and hemolysis are the clinical hallmarks of sickle cell disease which can lead to extramedullary hemopoises and reactive lymphadenopathy .However, rare cases of hematological malignancies have been reported in patients with sickle cell disease but are often underdiagnosed(2). We report a case of non hodgkin lymphoma in an adult patient with sickle cell anaemia that was associated with ahypercoaguble state.
CASE PRESENTATION: We report a 23-years old lady, who is a case of sickle cell disease and rheumatic heart disease. She presented with recurrent pulmonary emboli despite anticoagulation and left lower lobe pneumonia for which she required mechanical ventilation. CT chest showed hilar and mediastinal lymphadenpathy. Transbronchial fine needle aspiration were consistent with reactive lymphadenopathy. However , in view of atypical presentation and unresolving left lower lobe pneumonia leading to failure to wean off mechanical ventilation, true cut lung biopsy and transesophageal lymph node biopsy were performed and unmasked the diagnosis of non Hodgkin lymphoma( B cell type) for which she was started on chemotherapy.
DISCUSSION: In this case we report a rare association between sickle cell disease and non hodgkins lymphoma . Although extramedullary hematopoesis is a recognized cause of reactive lymphadenopathy in sickle cell disease, but when the lymph nodes are persistent and associated with other manifestation like fever ,weight loss and hypercoagulable state, other differential diagnosis needs to be entertained. These include infections especially tuberculosis, malignancy, lymphoproliferative and immunologic disorders. The association of tuberculosis with sickle has been suggested by Lionnet et al who reported higher incidence of lymph node tuberculosis in sickle cell disease than epidemiologically comparable population (3). In our community, tuberculosis is endemic especially in black and low socioeconomic class , therefore it was highly considered in our patient but seemed most unlikely due to repeatedly negative AFB stains in the cytology specimens and the negative culture .In adult SCD, only few cases of hematological malignancies have been reported in literature(2) These included acute leukemia, multiple myeloma ,Hodgkins disease and malignant histiocytosis. Up to our knowledge, our patient is the first case of non Hodgkin lymphoma reported in adult SCD. Although the relationship between these malignancies and sickle cell disease is not yet defined,the possibility of a common chromosomal alteration has been suggested,however ,further cytogenetic studies are still needed. The presentation of this case with recurrent PE involving main pulmonary arteries despite therapeutic anticoagulation was unusual.The association between hematological malignancies and hypercoagulable state is well known and is thought to to be related to factor V Leiden and prothrombin mutation,in addition to acquired antiphospholipid antibodies . However in diffuse large B-cell lymphoma the risk of thrombosis is much higher ( 35.7%).This is thought to result from local Compression of mediastinal vessels causing narrowing of the lumen and venous stasis
CONCLUSIONS: This case report highlights the importance of thoroughly investigating lymphadenopathy in sickle cell patients before accepting extramedullary hemopoeisis as the underlying cause, especially in presence of hypercoagulable state and unresolving pneumonia in order not to miss the possibility of lymphoma.
1) El-Hazmi MA, Warsy AS. On the nature of sickle cell disease in the south-western province of Saudi Arabia .Acta Haematol. 1986;76(4):212-6.
2) Paydas S: Sickle cell anemia and hematological neoplasias Leuk Lymphoma 2001 Jul;43(7):1431-4
3) Lionnet F., Bachmeyer C, Sloma I, Rossier A, Thioliere B, Maier M, Grateau G, Girot R: Tuberculosis in adult patients with Sickle cell disease J Infec 2007 Nov; 55(5): 439-44
DISCLOSURE: The following authors have nothing to disclose: Hadil AlOtair
No Product/Research Disclosure InformationKing Khalid University Hospital, Riyadh, Saudi Arabia