Chronic Lymphocytic Leukaemia Presenting with Pancytopenia: A Rare Haematological Coincidence

被引:0
|
作者
Jamal, Iffat [1 ]
Smita, Shuchi [1 ]
Raman, Ravi Bhushan [1 ]
Kumar, Kaushal [1 ]
Choudhary, Vijayanand [1 ]
机构
[1] Indira Gandhi Inst Med Sci, Dept Hematol Pathol, Patna, Bihar, India
关键词
Autoimmune; Cytopenias; Evans syndrome; Lymphocyte count; AUTOIMMUNE HEMOLYTIC-ANEMIA; FLUDARABINE; RITUXIMAB; CYCLOPHOSPHAMIDE; EXPRESSION; SURVIVAL;
D O I
10.7860/JCDR/2021/46541.14858
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Chronic Lymphocytic Leukaemia ( CLL) is commonly associated with autoimmune cytopenias but CLL presenting with pancytopenia is a rare occurrence. Here, we present a case of 56-year- old male patient, who presented with pallor, mild hepatosplenomegaly, right-sided pleural effusion in chest x-ray, pancytopenia and his bone marrow showed infiltration by mature appearing lymphoid cells making a diagnosis of CLL. Despite the frequency of these immune mediated cytopenia, only few cases of pancytopenia have been described so far to the best of our knowledge. Hence, to emphasise the rarity of such haematological coincidence we are presenting this case report. CLL generally presents with persistent absolute lymphocytosis on peripheral smear with an absolute monoclonal lymphocyte count of more than 5000/cumm. There is a common association with Autoimmune Haemolytic Anaemia (AIHA) and Immune Thrombocytopenia (ITP) and the association is called as Evan's syndrome. Its pathogenesis is associated with autoimmune process. AIHA, ITP, and Pure Red Cell Aplasia (PRCA) are commonly associated complications seen in CLL. The pathogenesis of AIHA and ITP are antibody this inplace of the reactions and abnormal T cell activity is noted in the pathogenesis of PRCA. Despite of the frequency of these immune mediated cytopenias noted in CLL, bone marrow was seen to be hypercellular in most of the cases and marrow hypoplasia have been reported in only 2 cases reported in literature so far. In the present case, although pancytopenia was present on peripheral blood smear examination but bone marrow was hypercellular for age with diffuse infiltration by monoclonal mature appearing lymphoid cells which makes this case all the more interesting. Despite repeated blood transfusions,antiviral drug Tenofovir and aggressive supportive measures for 6 months, he developed right-sided pleural effusion and succumbed to his illness.
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