Melioidosis with possible Haemophagocytic lymphohistiocytosis
View Article PDF
We report a case of Burkholderia pseudomallei bacteremia without pulmonary involvement, complicated by possible Haemophagocytic lymphohistiocytosis (HLH).
Melioidosis is a tropical disease caused by B. pseudomallei, a highly pathogenic gram-negative bacillus endemic in South-East Asia and Northern Australia.1–3 Transmission via percutaneous inoculation, aerosol inhalation and ingestion through contact with contaminated soil and water. Incubation period varies from 1–21 days.1–3 Clinical manifestations vary from sub-acute disease with localised skin lesions to fulminant septic shock.
Case report
A 47-year-old systems security engineer in Brunei was admitted one week after arriving in Auckland with two weeks of fevers, dry cough, lethargy and anorexia. There was no recent exposure to wet muddy outdoors. He had poorly controlled type 2 diabetes. Fever 38.8°C with tachycardia, and no focal signs of infection were noted. Laboratory investigations are shown in Table 1. Chest x-ray was normal. Cefuroxime and Metronidazole were started.
Table 1: Laboratory results of patient at baseline, day three, and at follow-up in four weeks.
MCV: Mean corpuscle volume; WBC: White cell count; CRP: C - reactive protein; ALT: Alanine transaminase; ALP: Alkaline phosphatase;GGT: Gamma glutaryl transpeptidase; APTT: Activated partial thromboplastin; HbA1c: Glycosylated haemoglobin; PR: Prothrombin ratio; ND*: Not done.
An ultrasound revealed multiple 5–10mm hypoechoic lesions suspicious of liver abscesses with no hepatosplenomegaly.
On day three, he developed hypotension (80/50mmHg) with ongoing high-grade fevers, worsening liver function, thrombocytopenia and an elevated ferritin (Table 1).
A CT abdomen showed new hepatosplenomegaly and confirmed multiple liver abscesses (Figures 1A and 1B), hypo-attenuated splenic lesions likely infarction, and ascending colitis. The combination of thrombocytopenia, high ferritin and hepatosplenomegaly raised the possibility of HLH. High dose prednisone (60mg) was started with early clinical response within 48 hours. Blood cultures were positive for gram-negative bacilli with bipolar staining. B. pseudomallei was identified and later confirmed with 16S rRNA.
Ceftazidime and Meropenem were commenced. Bone marrow biopsyraised possibility of HLH due to profound haemophagocytic activity (Figure 1C).
He responded well and completed total of four weeks of Ceftazidime after discharge on day 14. Prednisone was tapered over four weeks to reduce the risk of relapse. Follow-up confirmed ongoing improvement (Table 1), resolving splenomegaly and liver abscesses. Co-trimoxazole 960mg BD was given for three months in view of non-surgical management of liver abscesses, disseminated infection and concomitant Prednisone use.
Discussion
Melioidosis is rare in New Zealand.4 To our knowledge, this is the first case of extrapulmonary melioidosis with liver abscesses complicated with possible HLH. Melioidosis is commonly characterised by pneumonia and intra-organ abscesses with mortality rate of 40%.2,3 Recognised risk factors for melioidosis are diabetes, alcoholism, chronic lung disease and renal disease reflecting impairment in innate immune function.1–3 Longer antibiotic course (up to six months), including ‘induction’ with intravenous agents, is recommended for treatment.5
HLH is a life-threatening hyperinflammatory state mediated by impaired natural-killer (NK) and cytotoxic T cell functions. Laboratory features include pancytopenia; transaminitis; hyperferritinemia; hypofibrinogenemia or hypertriglyceridemia; high soluble IL -2 receptor levels; low/absent NK cells activity and haemophagocytosis on bone marrow biopsy (Henter et al Criteria 2004).6 HLH can be familial. Viral infections (commonly EBV, HIV), lymphoid malignancy, connective tissue diseases can also trigger HLH.7–9
Five out of eight Henter HLH criteria were fulfilled in our case. We could not perform high soluble IL -2 receptor, NK cells assay or molecular/genetic testing. Therefore, it cannot be confirmed whether this patient developed HLH or a syndrome mimicking HLH on the basis of cyptopenias, hepatosplenomegaly, high ferritin and bone marrow changes from severe melioidosis. High ferritin levels, although non-specific for HLH, may predict high mortality with levels >2000µg/L in non-malignancy related cases.11 Treatment of HLH is based on combination of early immunomodulators to suppress dysregulated immune response, directed treatment for underlying trigger/s, and good supportive care. Immunotherapeutic agents like steroids, etoposide, methotrexate and cyclosporine have been used.10
The outcome in our patient was excellent with multidisciplinary care, appropriate antibiotics and early use of steroids.
Figure 1:
1A and 1B: Coronal and Axial CT—showing hepatosplenomegaly with low attenuating cystic abscess collections. Area of wedge shaped hypoattentating area—splenic infarct.
1C: Bone marrow biopsy—showing erythoid phagocytosis.
Summary
Abstract
Aim
Method
Results
Conclusion
Author Information
Acknowledgements
Correspondence
Correspondence Email
Competing Interests
- Cheng AC, Currie BJ. Melioidosis: epidemiology, pathophysiology, and management. Clin Microbiol Rev. 2005; 18(2):383–416.
- Currie BJ, Ward L, Cheng AC. The epidemiology and clinical spectrum of melioidosis: 540 cases from the 20 year Darwin prospective study. PLoS Negl Trop Dis. 2010; 4(11):e900.
- Wiersinga WJ, Currie BJ, Peacock SJ. Melioidosis. N Engl J Med. 2012; 367(11):1035–44.
- Corkill MM, Cornere B. Melioidosis: a new disease to New Zealand. N Z Med J. 1987; 100(818):106–7.
- Currie B. Melioidosis: evolving concepts in epidemiology, pathogenesis, and treatment. Semin Respir Crit Care Med. 2015; 36(1):111–25.
- Ramos-Casals M, Brito-Zeron P, Lopez-Guillermo A, et al. Adult haemophagocytic syndrome. Lancet. 2014; 383(9927):1503–16.
- Maakaroun NR, Moanna A, Jacob JT, et al. Viral infections associated with haemophagocytic syndrome. Rev Med Virol. 2010; 20(2):93–105.
- Fardet L, Lambotte O, Meynard JL, et al. Reactive haemophagocytic syndrome in 58 HIV-1-infected patients: clinical features, underlying diseases and prognosis. AIDS. 2010; 24(9):1299–306.
- Murase T, Nakamura S, Kawauchi K, et al. An Asian variant of intravascular large B-cell lymphoma: clinical, pathological and cytogenetic approaches to diffuse large B-cell lymphoma associated with haemophagocytic syndrome. Br J Haematol. 2000; 111(3):826–34.
- Jordan M, Allen C, Weitzman S, et al. How I treat haemophagocytic lymphohistiocytosis. Blood. 2011; 118(15):4041–52.
- Grange S, Buchonnet G, Besnier E, et al. The use of ferritin to identify critically ill patients with secondary haemophagocytic lymphohistiocytosis. Crit Care Med. 2016; 44:e1045–1053.
For the PDF of this article,
contact nzmj@nzma.org.nz
Melioidosis with possible Haemophagocytic lymphohistiocytosis
View Article PDF
We report a case of Burkholderia pseudomallei bacteremia without pulmonary involvement, complicated by possible Haemophagocytic lymphohistiocytosis (HLH).
Melioidosis is a tropical disease caused by B. pseudomallei, a highly pathogenic gram-negative bacillus endemic in South-East Asia and Northern Australia.1–3 Transmission via percutaneous inoculation, aerosol inhalation and ingestion through contact with contaminated soil and water. Incubation period varies from 1–21 days.1–3 Clinical manifestations vary from sub-acute disease with localised skin lesions to fulminant septic shock.
Case report
A 47-year-old systems security engineer in Brunei was admitted one week after arriving in Auckland with two weeks of fevers, dry cough, lethargy and anorexia. There was no recent exposure to wet muddy outdoors. He had poorly controlled type 2 diabetes. Fever 38.8°C with tachycardia, and no focal signs of infection were noted. Laboratory investigations are shown in Table 1. Chest x-ray was normal. Cefuroxime and Metronidazole were started.
Table 1: Laboratory results of patient at baseline, day three, and at follow-up in four weeks.
MCV: Mean corpuscle volume; WBC: White cell count; CRP: C - reactive protein; ALT: Alanine transaminase; ALP: Alkaline phosphatase;GGT: Gamma glutaryl transpeptidase; APTT: Activated partial thromboplastin; HbA1c: Glycosylated haemoglobin; PR: Prothrombin ratio; ND*: Not done.
An ultrasound revealed multiple 5–10mm hypoechoic lesions suspicious of liver abscesses with no hepatosplenomegaly.
On day three, he developed hypotension (80/50mmHg) with ongoing high-grade fevers, worsening liver function, thrombocytopenia and an elevated ferritin (Table 1).
A CT abdomen showed new hepatosplenomegaly and confirmed multiple liver abscesses (Figures 1A and 1B), hypo-attenuated splenic lesions likely infarction, and ascending colitis. The combination of thrombocytopenia, high ferritin and hepatosplenomegaly raised the possibility of HLH. High dose prednisone (60mg) was started with early clinical response within 48 hours. Blood cultures were positive for gram-negative bacilli with bipolar staining. B. pseudomallei was identified and later confirmed with 16S rRNA.
Ceftazidime and Meropenem were commenced. Bone marrow biopsyraised possibility of HLH due to profound haemophagocytic activity (Figure 1C).
He responded well and completed total of four weeks of Ceftazidime after discharge on day 14. Prednisone was tapered over four weeks to reduce the risk of relapse. Follow-up confirmed ongoing improvement (Table 1), resolving splenomegaly and liver abscesses. Co-trimoxazole 960mg BD was given for three months in view of non-surgical management of liver abscesses, disseminated infection and concomitant Prednisone use.
Discussion
Melioidosis is rare in New Zealand.4 To our knowledge, this is the first case of extrapulmonary melioidosis with liver abscesses complicated with possible HLH. Melioidosis is commonly characterised by pneumonia and intra-organ abscesses with mortality rate of 40%.2,3 Recognised risk factors for melioidosis are diabetes, alcoholism, chronic lung disease and renal disease reflecting impairment in innate immune function.1–3 Longer antibiotic course (up to six months), including ‘induction’ with intravenous agents, is recommended for treatment.5
HLH is a life-threatening hyperinflammatory state mediated by impaired natural-killer (NK) and cytotoxic T cell functions. Laboratory features include pancytopenia; transaminitis; hyperferritinemia; hypofibrinogenemia or hypertriglyceridemia; high soluble IL -2 receptor levels; low/absent NK cells activity and haemophagocytosis on bone marrow biopsy (Henter et al Criteria 2004).6 HLH can be familial. Viral infections (commonly EBV, HIV), lymphoid malignancy, connective tissue diseases can also trigger HLH.7–9
Five out of eight Henter HLH criteria were fulfilled in our case. We could not perform high soluble IL -2 receptor, NK cells assay or molecular/genetic testing. Therefore, it cannot be confirmed whether this patient developed HLH or a syndrome mimicking HLH on the basis of cyptopenias, hepatosplenomegaly, high ferritin and bone marrow changes from severe melioidosis. High ferritin levels, although non-specific for HLH, may predict high mortality with levels >2000µg/L in non-malignancy related cases.11 Treatment of HLH is based on combination of early immunomodulators to suppress dysregulated immune response, directed treatment for underlying trigger/s, and good supportive care. Immunotherapeutic agents like steroids, etoposide, methotrexate and cyclosporine have been used.10
The outcome in our patient was excellent with multidisciplinary care, appropriate antibiotics and early use of steroids.
Figure 1:
1A and 1B: Coronal and Axial CT—showing hepatosplenomegaly with low attenuating cystic abscess collections. Area of wedge shaped hypoattentating area—splenic infarct.
1C: Bone marrow biopsy—showing erythoid phagocytosis.
Summary
Abstract
Aim
Method
Results
Conclusion
Author Information
Acknowledgements
Correspondence
Correspondence Email
Competing Interests
- Cheng AC, Currie BJ. Melioidosis: epidemiology, pathophysiology, and management. Clin Microbiol Rev. 2005; 18(2):383–416.
- Currie BJ, Ward L, Cheng AC. The epidemiology and clinical spectrum of melioidosis: 540 cases from the 20 year Darwin prospective study. PLoS Negl Trop Dis. 2010; 4(11):e900.
- Wiersinga WJ, Currie BJ, Peacock SJ. Melioidosis. N Engl J Med. 2012; 367(11):1035–44.
- Corkill MM, Cornere B. Melioidosis: a new disease to New Zealand. N Z Med J. 1987; 100(818):106–7.
- Currie B. Melioidosis: evolving concepts in epidemiology, pathogenesis, and treatment. Semin Respir Crit Care Med. 2015; 36(1):111–25.
- Ramos-Casals M, Brito-Zeron P, Lopez-Guillermo A, et al. Adult haemophagocytic syndrome. Lancet. 2014; 383(9927):1503–16.
- Maakaroun NR, Moanna A, Jacob JT, et al. Viral infections associated with haemophagocytic syndrome. Rev Med Virol. 2010; 20(2):93–105.
- Fardet L, Lambotte O, Meynard JL, et al. Reactive haemophagocytic syndrome in 58 HIV-1-infected patients: clinical features, underlying diseases and prognosis. AIDS. 2010; 24(9):1299–306.
- Murase T, Nakamura S, Kawauchi K, et al. An Asian variant of intravascular large B-cell lymphoma: clinical, pathological and cytogenetic approaches to diffuse large B-cell lymphoma associated with haemophagocytic syndrome. Br J Haematol. 2000; 111(3):826–34.
- Jordan M, Allen C, Weitzman S, et al. How I treat haemophagocytic lymphohistiocytosis. Blood. 2011; 118(15):4041–52.
- Grange S, Buchonnet G, Besnier E, et al. The use of ferritin to identify critically ill patients with secondary haemophagocytic lymphohistiocytosis. Crit Care Med. 2016; 44:e1045–1053.
For the PDF of this article,
contact nzmj@nzma.org.nz
Melioidosis with possible Haemophagocytic lymphohistiocytosis
View Article PDF
We report a case of Burkholderia pseudomallei bacteremia without pulmonary involvement, complicated by possible Haemophagocytic lymphohistiocytosis (HLH).
Melioidosis is a tropical disease caused by B. pseudomallei, a highly pathogenic gram-negative bacillus endemic in South-East Asia and Northern Australia.1–3 Transmission via percutaneous inoculation, aerosol inhalation and ingestion through contact with contaminated soil and water. Incubation period varies from 1–21 days.1–3 Clinical manifestations vary from sub-acute disease with localised skin lesions to fulminant septic shock.
Case report
A 47-year-old systems security engineer in Brunei was admitted one week after arriving in Auckland with two weeks of fevers, dry cough, lethargy and anorexia. There was no recent exposure to wet muddy outdoors. He had poorly controlled type 2 diabetes. Fever 38.8°C with tachycardia, and no focal signs of infection were noted. Laboratory investigations are shown in Table 1. Chest x-ray was normal. Cefuroxime and Metronidazole were started.
Table 1: Laboratory results of patient at baseline, day three, and at follow-up in four weeks.
MCV: Mean corpuscle volume; WBC: White cell count; CRP: C - reactive protein; ALT: Alanine transaminase; ALP: Alkaline phosphatase;GGT: Gamma glutaryl transpeptidase; APTT: Activated partial thromboplastin; HbA1c: Glycosylated haemoglobin; PR: Prothrombin ratio; ND*: Not done.
An ultrasound revealed multiple 5–10mm hypoechoic lesions suspicious of liver abscesses with no hepatosplenomegaly.
On day three, he developed hypotension (80/50mmHg) with ongoing high-grade fevers, worsening liver function, thrombocytopenia and an elevated ferritin (Table 1).
A CT abdomen showed new hepatosplenomegaly and confirmed multiple liver abscesses (Figures 1A and 1B), hypo-attenuated splenic lesions likely infarction, and ascending colitis. The combination of thrombocytopenia, high ferritin and hepatosplenomegaly raised the possibility of HLH. High dose prednisone (60mg) was started with early clinical response within 48 hours. Blood cultures were positive for gram-negative bacilli with bipolar staining. B. pseudomallei was identified and later confirmed with 16S rRNA.
Ceftazidime and Meropenem were commenced. Bone marrow biopsyraised possibility of HLH due to profound haemophagocytic activity (Figure 1C).
He responded well and completed total of four weeks of Ceftazidime after discharge on day 14. Prednisone was tapered over four weeks to reduce the risk of relapse. Follow-up confirmed ongoing improvement (Table 1), resolving splenomegaly and liver abscesses. Co-trimoxazole 960mg BD was given for three months in view of non-surgical management of liver abscesses, disseminated infection and concomitant Prednisone use.
Discussion
Melioidosis is rare in New Zealand.4 To our knowledge, this is the first case of extrapulmonary melioidosis with liver abscesses complicated with possible HLH. Melioidosis is commonly characterised by pneumonia and intra-organ abscesses with mortality rate of 40%.2,3 Recognised risk factors for melioidosis are diabetes, alcoholism, chronic lung disease and renal disease reflecting impairment in innate immune function.1–3 Longer antibiotic course (up to six months), including ‘induction’ with intravenous agents, is recommended for treatment.5
HLH is a life-threatening hyperinflammatory state mediated by impaired natural-killer (NK) and cytotoxic T cell functions. Laboratory features include pancytopenia; transaminitis; hyperferritinemia; hypofibrinogenemia or hypertriglyceridemia; high soluble IL -2 receptor levels; low/absent NK cells activity and haemophagocytosis on bone marrow biopsy (Henter et al Criteria 2004).6 HLH can be familial. Viral infections (commonly EBV, HIV), lymphoid malignancy, connective tissue diseases can also trigger HLH.7–9
Five out of eight Henter HLH criteria were fulfilled in our case. We could not perform high soluble IL -2 receptor, NK cells assay or molecular/genetic testing. Therefore, it cannot be confirmed whether this patient developed HLH or a syndrome mimicking HLH on the basis of cyptopenias, hepatosplenomegaly, high ferritin and bone marrow changes from severe melioidosis. High ferritin levels, although non-specific for HLH, may predict high mortality with levels >2000µg/L in non-malignancy related cases.11 Treatment of HLH is based on combination of early immunomodulators to suppress dysregulated immune response, directed treatment for underlying trigger/s, and good supportive care. Immunotherapeutic agents like steroids, etoposide, methotrexate and cyclosporine have been used.10
The outcome in our patient was excellent with multidisciplinary care, appropriate antibiotics and early use of steroids.
Figure 1:
1A and 1B: Coronal and Axial CT—showing hepatosplenomegaly with low attenuating cystic abscess collections. Area of wedge shaped hypoattentating area—splenic infarct.
1C: Bone marrow biopsy—showing erythoid phagocytosis.
Summary
Abstract
Aim
Method
Results
Conclusion
Author Information
Acknowledgements
Correspondence
Correspondence Email
Competing Interests
- Cheng AC, Currie BJ. Melioidosis: epidemiology, pathophysiology, and management. Clin Microbiol Rev. 2005; 18(2):383–416.
- Currie BJ, Ward L, Cheng AC. The epidemiology and clinical spectrum of melioidosis: 540 cases from the 20 year Darwin prospective study. PLoS Negl Trop Dis. 2010; 4(11):e900.
- Wiersinga WJ, Currie BJ, Peacock SJ. Melioidosis. N Engl J Med. 2012; 367(11):1035–44.
- Corkill MM, Cornere B. Melioidosis: a new disease to New Zealand. N Z Med J. 1987; 100(818):106–7.
- Currie B. Melioidosis: evolving concepts in epidemiology, pathogenesis, and treatment. Semin Respir Crit Care Med. 2015; 36(1):111–25.
- Ramos-Casals M, Brito-Zeron P, Lopez-Guillermo A, et al. Adult haemophagocytic syndrome. Lancet. 2014; 383(9927):1503–16.
- Maakaroun NR, Moanna A, Jacob JT, et al. Viral infections associated with haemophagocytic syndrome. Rev Med Virol. 2010; 20(2):93–105.
- Fardet L, Lambotte O, Meynard JL, et al. Reactive haemophagocytic syndrome in 58 HIV-1-infected patients: clinical features, underlying diseases and prognosis. AIDS. 2010; 24(9):1299–306.
- Murase T, Nakamura S, Kawauchi K, et al. An Asian variant of intravascular large B-cell lymphoma: clinical, pathological and cytogenetic approaches to diffuse large B-cell lymphoma associated with haemophagocytic syndrome. Br J Haematol. 2000; 111(3):826–34.
- Jordan M, Allen C, Weitzman S, et al. How I treat haemophagocytic lymphohistiocytosis. Blood. 2011; 118(15):4041–52.
- Grange S, Buchonnet G, Besnier E, et al. The use of ferritin to identify critically ill patients with secondary haemophagocytic lymphohistiocytosis. Crit Care Med. 2016; 44:e1045–1053.
Contact diana@nzma.org.nz
for the PDF of this article
Melioidosis with possible Haemophagocytic lymphohistiocytosis
View Article PDF
We report a case of Burkholderia pseudomallei bacteremia without pulmonary involvement, complicated by possible Haemophagocytic lymphohistiocytosis (HLH).
Melioidosis is a tropical disease caused by B. pseudomallei, a highly pathogenic gram-negative bacillus endemic in South-East Asia and Northern Australia.1–3 Transmission via percutaneous inoculation, aerosol inhalation and ingestion through contact with contaminated soil and water. Incubation period varies from 1–21 days.1–3 Clinical manifestations vary from sub-acute disease with localised skin lesions to fulminant septic shock.
Case report
A 47-year-old systems security engineer in Brunei was admitted one week after arriving in Auckland with two weeks of fevers, dry cough, lethargy and anorexia. There was no recent exposure to wet muddy outdoors. He had poorly controlled type 2 diabetes. Fever 38.8°C with tachycardia, and no focal signs of infection were noted. Laboratory investigations are shown in Table 1. Chest x-ray was normal. Cefuroxime and Metronidazole were started.
Table 1: Laboratory results of patient at baseline, day three, and at follow-up in four weeks.
MCV: Mean corpuscle volume; WBC: White cell count; CRP: C - reactive protein; ALT: Alanine transaminase; ALP: Alkaline phosphatase;GGT: Gamma glutaryl transpeptidase; APTT: Activated partial thromboplastin; HbA1c: Glycosylated haemoglobin; PR: Prothrombin ratio; ND*: Not done.
An ultrasound revealed multiple 5–10mm hypoechoic lesions suspicious of liver abscesses with no hepatosplenomegaly.
On day three, he developed hypotension (80/50mmHg) with ongoing high-grade fevers, worsening liver function, thrombocytopenia and an elevated ferritin (Table 1).
A CT abdomen showed new hepatosplenomegaly and confirmed multiple liver abscesses (Figures 1A and 1B), hypo-attenuated splenic lesions likely infarction, and ascending colitis. The combination of thrombocytopenia, high ferritin and hepatosplenomegaly raised the possibility of HLH. High dose prednisone (60mg) was started with early clinical response within 48 hours. Blood cultures were positive for gram-negative bacilli with bipolar staining. B. pseudomallei was identified and later confirmed with 16S rRNA.
Ceftazidime and Meropenem were commenced. Bone marrow biopsyraised possibility of HLH due to profound haemophagocytic activity (Figure 1C).
He responded well and completed total of four weeks of Ceftazidime after discharge on day 14. Prednisone was tapered over four weeks to reduce the risk of relapse. Follow-up confirmed ongoing improvement (Table 1), resolving splenomegaly and liver abscesses. Co-trimoxazole 960mg BD was given for three months in view of non-surgical management of liver abscesses, disseminated infection and concomitant Prednisone use.
Discussion
Melioidosis is rare in New Zealand.4 To our knowledge, this is the first case of extrapulmonary melioidosis with liver abscesses complicated with possible HLH. Melioidosis is commonly characterised by pneumonia and intra-organ abscesses with mortality rate of 40%.2,3 Recognised risk factors for melioidosis are diabetes, alcoholism, chronic lung disease and renal disease reflecting impairment in innate immune function.1–3 Longer antibiotic course (up to six months), including ‘induction’ with intravenous agents, is recommended for treatment.5
HLH is a life-threatening hyperinflammatory state mediated by impaired natural-killer (NK) and cytotoxic T cell functions. Laboratory features include pancytopenia; transaminitis; hyperferritinemia; hypofibrinogenemia or hypertriglyceridemia; high soluble IL -2 receptor levels; low/absent NK cells activity and haemophagocytosis on bone marrow biopsy (Henter et al Criteria 2004).6 HLH can be familial. Viral infections (commonly EBV, HIV), lymphoid malignancy, connective tissue diseases can also trigger HLH.7–9
Five out of eight Henter HLH criteria were fulfilled in our case. We could not perform high soluble IL -2 receptor, NK cells assay or molecular/genetic testing. Therefore, it cannot be confirmed whether this patient developed HLH or a syndrome mimicking HLH on the basis of cyptopenias, hepatosplenomegaly, high ferritin and bone marrow changes from severe melioidosis. High ferritin levels, although non-specific for HLH, may predict high mortality with levels >2000µg/L in non-malignancy related cases.11 Treatment of HLH is based on combination of early immunomodulators to suppress dysregulated immune response, directed treatment for underlying trigger/s, and good supportive care. Immunotherapeutic agents like steroids, etoposide, methotrexate and cyclosporine have been used.10
The outcome in our patient was excellent with multidisciplinary care, appropriate antibiotics and early use of steroids.
Figure 1:
1A and 1B: Coronal and Axial CT—showing hepatosplenomegaly with low attenuating cystic abscess collections. Area of wedge shaped hypoattentating area—splenic infarct.
1C: Bone marrow biopsy—showing erythoid phagocytosis.
Summary
Abstract
Aim
Method
Results
Conclusion
Author Information
Acknowledgements
Correspondence
Correspondence Email
Competing Interests
- Cheng AC, Currie BJ. Melioidosis: epidemiology, pathophysiology, and management. Clin Microbiol Rev. 2005; 18(2):383–416.
- Currie BJ, Ward L, Cheng AC. The epidemiology and clinical spectrum of melioidosis: 540 cases from the 20 year Darwin prospective study. PLoS Negl Trop Dis. 2010; 4(11):e900.
- Wiersinga WJ, Currie BJ, Peacock SJ. Melioidosis. N Engl J Med. 2012; 367(11):1035–44.
- Corkill MM, Cornere B. Melioidosis: a new disease to New Zealand. N Z Med J. 1987; 100(818):106–7.
- Currie B. Melioidosis: evolving concepts in epidemiology, pathogenesis, and treatment. Semin Respir Crit Care Med. 2015; 36(1):111–25.
- Ramos-Casals M, Brito-Zeron P, Lopez-Guillermo A, et al. Adult haemophagocytic syndrome. Lancet. 2014; 383(9927):1503–16.
- Maakaroun NR, Moanna A, Jacob JT, et al. Viral infections associated with haemophagocytic syndrome. Rev Med Virol. 2010; 20(2):93–105.
- Fardet L, Lambotte O, Meynard JL, et al. Reactive haemophagocytic syndrome in 58 HIV-1-infected patients: clinical features, underlying diseases and prognosis. AIDS. 2010; 24(9):1299–306.
- Murase T, Nakamura S, Kawauchi K, et al. An Asian variant of intravascular large B-cell lymphoma: clinical, pathological and cytogenetic approaches to diffuse large B-cell lymphoma associated with haemophagocytic syndrome. Br J Haematol. 2000; 111(3):826–34.
- Jordan M, Allen C, Weitzman S, et al. How I treat haemophagocytic lymphohistiocytosis. Blood. 2011; 118(15):4041–52.
- Grange S, Buchonnet G, Besnier E, et al. The use of ferritin to identify critically ill patients with secondary haemophagocytic lymphohistiocytosis. Crit Care Med. 2016; 44:e1045–1053.
Contact diana@nzma.org.nz
for the PDF of this article
Melioidosis with possible Haemophagocytic lymphohistiocytosis
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