Hemophagocytic Lymphohistiocytosis (HLH) and Sarcoidosis: A Complex Case Study
Dr Prajna Anirvan
Department of Gastroenterology
SCB Medical College, Cuttack
India.
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24-year old unmarried male, mason by occupation, diagnosed with
cutaneous sarcoidosis 9 months ago, on maintenance steroids in
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No history of diabetes, alcohol intake, or IV drug abuse
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Jaundice
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Fever
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Referral due to non-improvement
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No history suggestive of cholestasis/nausea/vomiting/alternative medicine intake.
Dragging sensation in right upper abdomen
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High grade continuous fever without chill and rigor, subsided on taking antipyretics
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BMI 23 kg/m², Icterus, Temperature: 102°F
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Abdomen: Liver 18 cm, firm, non-tender, spleen enlarged 2 cm below LCM, no free fluid
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USG abdomen –
Hepatomegaly (21 cm) with Grade 2 Fatty Changes, IHBR
normal, no SOL, Splenomegaly, Few mildly enlarged porta
hepatic nodes (13 mm), Portal vein - not dilated.
MRI abdomen with MRCP –
Hepatomegaly, no SOL, IHBR normal
Splenomegaly
UGI endoscopy:
Normal mucosal study till D2, no varices
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HAV/HEV IgM: non reactive
HbsAg, HCV, Anti-HBc Total - negative
CMV, EBV, HIV, COVID-19 RTPCR‐ Negative
Malaria/Dengue: Neg
Blood, Urine Cultures : Sterile
Procalcitonin: 0.2
μ
g/L
ANA- negative, AIH profile- negative
Mantoux Test- Non reactive, IGRA - negative
? Sarcoidosis with hepatic involvement not
responding to steroids
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High spiking fever
Jaundice
Oral steroids increased to 40 mg/day, antibiotics started, not responding.
Liver biopsy and bone marrow biopsy done.
Is it
Secondary
Hemophagocytic
Lymphohistiocytosis(HLH)/
Macrophage Activation
Syndrome(MAS)???
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IV Methylprednisolone 1 gm/day pulse dose started for treatment of HLH.
Patient afebrile at Day 3.
Oral cyclosporine at 2 mg/kg started.
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Grade II fatty liver, liver span-15 cm
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How commonly is sarcoidosis complicated by HLH/MAS?
Was hepatosplenomegaly due to HLH
per se
or due to sarcoidosis involving the liver?
How to distinguish hepatic sarcoidosis from HLH in the background of deranged LFT?
Did low dose steroid (10 mg/day) which patient was taking suppress HLH from
manifesting initially?
This case study delves into a challenging scenario where a hepatologist grapples with a patient presenting HLH associated with liver injury in the context of sarcoidosis. Despite inconclusive initial investigations, further work-up reveals indications of secondary HLH/MAS. The management involves a careful balance of treatments to address both conditions, raising questions on the differentiation of hepatic sarcoidosis from HLH and the efficacy of low-dose steroids in suppressing HLH.
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Presentation Transcript
Preceptorship Programme 23 to 25 May 2022 Case: Hemophagocytic Lymphohistiocytosis (HLH) associated with liver injury in Sarcoidosis hepatologist on the horns of a dilemma Dr Prajna Anirvan Department of Gastroenterology SCB Medical College, Cuttack India.
Initial Labs and Provisional Diagnosis Parameter Value Hemoglobin (gm/dL) 11 USG abdomen Hepatomegaly (21 cm) with Grade 2 Fatty Changes, IHBR normal, no SOL, Splenomegaly, Few mildly enlarged porta hepatic nodes (13 mm), Portal vein - not dilated. MRI abdomen with MRCP Hepatomegaly, no SOL, IHBR normal Splenomegaly UGI endoscopy: Normal mucosal study till D2, no varices ESR (mm AEFH)/ CRP (mg/L) Total leukocyte Count (x10 /mm ) Platelet Count (x10 /mm ) 6 38 2.8 210 Total Bilirubin/Direct Bilirubin (mg/dL) AST (IU/L) 7.2/4.1 Etiological work up HAV/HEV IgM: non reactive HbsAg, HCV, Anti-HBc Total - negative CMV, EBV, HIV, COVID-19 RTPCR Negative Malaria/Dengue: Neg Blood, Urine Cultures : Sterile Procalcitonin: 0.2 g/L ANA- negative, AIH profile- negative Mantoux Test- Non reactive, IGRA - negative 727 ALT (IU/L) 606 ALP (IU/L) 817 Albumin/globulin (gm/dL) 3.1/3.0 INR 1.29 Creatinine (mg/dL) 0.7 ? Sarcoidosis with hepatic involvement not responding to steroids Na/K (mmol/L) 128/4.3 Fasting Blood Sugar (mg/dL) 89
Clinical Course and Further Work up Parameter Serum triglyceride (mg/dL) Serum ferritin ( g/L) Serum LDH (IU/L) Value Reference range 45-150 1463 Is it In view of raised CRP and normal ESR Secondary Hemophagocytic Lymphohistiocytosis(HLH)/ Macrophage Activation Syndrome(MAS)??? 13-400 >2000 200-450 1811 Serum fibrinogen (mg/dL) 110-420 73.6
Management Day 7 Parameter Bilirubin (mg/dL) AST (IU/L) ALT (IU/L) ALP (IU/L) Bilirubin (mg/dL) AST (IU/L) ALT (IU/L) ALP (IU/L) Value 7.1 267 231 750 3.9 76 81 510 Parameter Bilirubin(mg/dL) Value 0.6 Day 90 AST(IU/L) 67 ALT 53 ALP 245 Day 30 USG(abdomen)- Grade II fatty liver, liver span-15 cm
Questions How commonly is sarcoidosis complicated by HLH/MAS? Was hepatosplenomegaly due to HLH per se or due to sarcoidosis involving the liver? How to distinguish hepatic sarcoidosis from HLH in the background of deranged LFT? Did low dose steroid (10 mg/day) which patient was taking suppress HLH from manifesting initially?
