Author(s): ,
Thinzar Ko Ko
Specialist Registrar (Haematology),Kings College Hospital,London,United Kingdom
Henry Wood
Specialist Registrar (Haematology),Kings College Hospital,London,United Kingdom
Sameer Patel
Consultant (Critical care, Liver intensive care & ECMO),Kings College Hospital,London,United Kingdom
Tasneem Pirani
Consultant (Critical care, Liver intensive care & ECMO),Kings College Hospital,London,United Kingdom
Shireen Kassam
Consultant Haematologist (Haematology),Kings College Hospital,London,United Kingdom
EHA Library. Ko Ko T. Jun 15, 2019; 267129; PS1512
Dr. Thinzar Ko Ko
Dr. Thinzar Ko Ko

Abstract: PS1512

Type: Poster Presentation

Presentation during EHA24: On Saturday, June 15, 2019 from 17:30 - 19:00

Location: Poster area

Haematological diagnoses can be associated with liver failure. Cytopenias are also common in patients presenting with liver failure and following liver transplantation. Distinguishing a primary haematological/immunological cause from secondary causes can be difficult and a bone marrow biopsy (BMB) remains a frequently performed investigation.

To evaluate the diagnostic utility of a BMB and its influence on patients management in the UK’s largest liver unit

We retrospectively collected clinical, biochemical and pathological data on consecutives referrals to the haematology department for BMB in patients with liver failure or a liver transplant between October 2012 and December 2018. Both paper and electronic patient records were used. We defined cytopenias as Hb <100g/l, platelets <100x109/l and neutrophils <1.8x109/l present in more than 2 cell lines.

108 BMBs were performed in 103 patients. Five patients had two BMBs. The median age was 47.7 years (range 18-76 years) with 57 (55%) male patients. Forty-three (42%) patients had received a liver transplant.

The indications for BMB were possible haemangiolymphophagocytosis (HLH) in 28 (27%), cytopenias in 47 (46%), post-transplant lymphoproliferative disorder (PTLD) in 14 (14%), myeloproliferative neoplasm (MPN) in 10 (10%) patients with Budd-Chiari syndrome, myeloma in 2 (2%), lymphoma in 1 (1%) and storage disorder in 1 (1%).

The final diagnosis after BMB included HLH in 8 (8%) patients, hepatosplenic T-cell lymphoma in 2 (2%), T-cell large granular leukaemia (T-LGL) in 1 (1%), aplastic anaemia in 4 (4%), telemeropathy in 2 (2%), probable MDS in 4 (4%), immune thrombocytopenia in 1 (1%), and MPN in 4 (4%). All 4 with MPN had evidence of the JAK2 V617F mutation. The BMB did not inform the final diagnosis in 77 (75%) patients.

In those who were confirmed to have HLH, the trigger was likely viral with CMV and/or EBV viraemia in 6, EBV and HSV viraemia in 1 and HHV8 viraemia in 1.  Four patients were treated actively with various combinations of ganciclovir, rituximab, IVIG, steroids and etoposide, however, 6/8 patients died.

In 4 patients diagnosed with aplastic anaemia, 1 patient was treated with ciclosporin followed by allogeneic stem cell transplant (SCT), 1 patient is awaiting a SCT and 2 patients are under active surveillance only. In the 4 patients with probable MDS, 1 was treated with supportive (GCSF and erythropoietin), 2 are under observation and 1 has died. The 2 patients with telomeropathy, 1 with immune thrombocytopenia and 1 with T-LGL are all under active surveillance. Both patients with hepatosplenic T-cell lymphoma have died; 1 prior to chemotherapy and 1 patient died with from relapsed disease.

Although we did not diagnose PTLD from the BMB, 8 (8%) patients subsequently received this diagnosis from another biopsy site. Four of the 8 patients have died. In the 4 patients diagnosed with an MPN, all 4 patients (4%) were treated with hydroxycarbamide. The 5 patients who had repeat BMBs were referred for possible HLH of which the diagnosis was subsequently made in 4. Overall, 42 (41%) patients have died.

Although the majority of BMBs (75%) did not reveal a diagnosis, a haematological/immunological diagnosis was made in 25% of referrals. Therefore, a BMB remains an important investigation in patients with liver failure and cytopenias with a wide range of potential diagnoses that have implications for therapy. 

Session topic: 35. Quality of life, palliative & supportive care, ethics and health economics

Keyword(s): Bone marrow biopsy, Diagnosis, Liver disease

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