Forme fruste of HLH (Haemophagocytic lymphohistiocytosis): Diagnostic and therapeutic challenges

BMJ Case Reports

Volumn 2015, Issue , 2015, Pages

  Inoue, Susumu ^a   Mangat, Chetna ^a   Rafe'e, Yaseen ^a   Sharman, Mahesh ^a  

^a Hurley Children's Hospital   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLSALICYLIC ACID; ANTIBIOTIC AGENT; ANTIPYRETIC AGENT; C REACTIVE PROTEIN; DRUG THERAPY; FERRITIN; FIBRINOGEN; FUNGUS ANTIGEN; IMMUNOGLOBULIN; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M; INTERLEUKIN 2 RECEPTOR; LACTATE DEHYDROGENASE; PERFORIN; TRIACYLGLYCEROL;

ADENOVIRIDAE; ANCA ASSOCIATED VASCULITIS; ARTICLE; AUTOIMMUNE DISEASE; BONE MARROW BIOPSY; CASE REPORT; CELL NUCLEUS; CEREBROSPINAL FLUID; CHEDIAK HIGASHI SYNDROME; CHILD; COMPLICATION; CONJUNCTIVA DISEASE; COUGHING; CYTOKINE STORM; DIAGNOSIS; DIARRHEA; DIFFERENTIAL DIAGNOSIS; DISEASE SEVERITY; DRUG THERAPY; ECHOCARDIOGRAPHY; ENTEROVIRUS; EPSTEIN BARR VIRUS; ERYTHEMA; ERYTHROCYTE SEDIMENTATION RATE; ERYTHROPOIESIS; ETIOLOGY; FECES ANALYSIS; FEMALE; FEVER; FOLLOW UP; GENETIC SCREENING; GENETICS; GRISCELLI SYNDROME; HEMOPHAGOCYTIC SYNDROME; HEPATOMEGALY; HISTIOCYTE; HISTOPLASMA; HOSPITALIZATION; HUMAN; HUMAN CELL; HUMAN PARVOVIRUS B19; HUMAN TISSUE; HYPERTRIGLYCIDEMIA; HYPOFIBRINOGENEMIA; INFANT; INFLAMMATORY BOWEL DISEASE; INFLUENZA A; INFLUENZA B; IRRITABILITY; JUVENILE RHEUMATOID ARTHRITIS; LEGIONELLA; LISTERIA MONOCYTOGENES; LOW DRUG DOSE; LYMPHADENOPATHY; LYMPHOHISTIOCYTOSIS, HEMOPHAGOCYTIC; MEVALONIC ACIDURIA; MOUTH DISEASE; MUCOCUTANEOUS LYMPH NODE SYNDROME; NEUTROPHIL COUNT; NONHUMAN; OCULAR ALBINISM; OTALGIA; PARTIAL THROMBOPLASTIN TIME; PATHOPHYSIOLOGY; PERINEAL ERYTHEMA; PHAGOCYTOSIS; POLYARTERITIS NODOSA; POLYMERASE CHAIN REACTION; PREDICTION; PRESCHOOL CHILD; PRF 1 GENE; PROTHROMBIN TIME; RAPID TEST; RASH; RECURRENT DISEASE; RETICULOCYTE; RHINOVIRUS; SPLENOMEGALY; STX11 GENE; STXBP2 GENE; TREATMENT DURATION; TREATMENT OUTCOME; UNC13D GENE; VOMITING;

COMPLICATIONS; DIAGNOSIS; DIAGNOSIS, DIFFERENTIAL; DRUG THERAPY; ETIOLOGY; FEMALE; FEVER; GENETIC TESTING; GENETICS; HUMANS; IMMUNOGLOBULINS, INTRAVENOUS; INFANT; LYMPHOHISTIOCYTOSIS, HEMOPHAGOCYTIC; MUCOCUTANEOUS LYMPH NODE SYNDROME; RECURRENCE; THERAPEUTIC USE;

EID: 84964285574     PISSN: None     EISSN: 1757790X     Source Type: Journal    
DOI: 10.1136/bcr-2014-206190     Document Type: Article

References (22)

1. Smith RM, Jones RB, Jane DW Progress in treatment of ANCA associated vasculitis. Arthritis Res Ther 2012; 14:210.
2. Thors VS, Vastert SJ, Wulffraat N, et al Periodic fever in MVK deficiency: a patient initially diagnosed with incomplete Kawasaki disease. Pediatr 2014; 133:e461-5.
3. Dajani AS, Taubert KA, Gerber M.A., et al. Diagnosis and therapy of Kawasaki disease in children. Circulation 1993; 87:1776-80.
4. Janka GE, Lehmberg K. Hemophagocytic lymphohistiocytosis: pathogenesis and treatment. Hematology Am Soc Hematol Educ Program 2013; 2013:605-11.
5. Chandrakasan S, Filipovich AH Hemophagocytic lymphohistiocytosis: advances in pathophysiology, diagnosis, and treatment. J Pediatr 2013; 163:1253-9.
6. Lin CI, Yu HH, Lee J.H., et al. Clinical analysis of macrophage activation syndrome in paediatric patients with autoimmune disease. Clin Rheumatol 2012; 31:1223-30.
7. Alvarez-Cardona A., Rodriguez-Lozano AL, Blancas-Galicia L, et al. Intravenous immunoglobulin treatment for macrophage activation syndrome complicating chronic granulomatous disease. J Clin Immunol 2012; 32:207-11.
8. Imashuku S. Treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH); update 201. J Pediatr Hematol Oncol 2011; 33:35-9.
9. Hot A, Madoux MH, Viard J.P., et al. Successful treatment of cytomegalovirus-associated hemophagocytic syndrome by intravenous immunoglobulins. Am J Hematol 2008; 3:159-62.
10. Muise A, Tallett SE, Silverman ED Are children with Kawasaki disease and prolonged fever at risk for macrophage activation syndrome? Pediatr 2003; 112:e495-7.
11. Ohga S, Oohshima A Fukushige J, et al Histiocytic haemophagocytosis in a patient with Kawasaki disease: changes in the hypercytokinaemic state. Eur J Pediatr 1995; 154:539-41.
12. Titze U, Janka G, Schneider E.M., et al. Hemophagocytic lymphohistiocytosis and Kawasaki disease: combined manifestation and differential diagnosis. Pediatr Blood Cancer 2009; 53:493-5.
13. Kang H-R, Yong-hoon Kwon Y-H, Eun-Sun Yoo E-S, et al. Clinical characteristics of hemophagocytic lymphohistiocytosis following Kawasaki disease: differentiation from recurrent Kawasaki disease. Blood Res 2013; 48:254-7.
14. Chen Y, Shang S, Zhang C., et al. Hemophagocytic lymphohistiocytosis at initiation of Kawasaki disease and their differential diagnosis. Pediatr Heamtol Oncol 2010; 27:244-9.
15. Kim HK, Kim HG, Cho S.J., et al. Clinical characteristics of hemophagocytic lymphohistiocytosis related to Kawasaki disease. Pediatr Hematol Oncol 2011; 28:230-6.
16. Palazzi DL, McClain KL, Kaplan SL Hemophagocytic syndrome after Kawasaki disease. Pediatr Infect Dis J 2003; 22:663-5.
17. Hendricks M, Pillay S, Davidson S. et al Kawasaki disease preceding hemophagocytic lymphohistiocytosis: challenges for developing world practioners. Pediatr Blood Cancer 2010; 54:1023-5.
18. Simonini G, Paganini I, Innocenti L. Macrophage activation syndrome/hemophagocytic lymphohistiocytosis and Kawasaki disease. Pediatr Blood Cancer 2010; 55:591.
19. Niwa K, Aotsuka H, Hamada H., et al. Thrombocytopenia: a risk factor for acute myocardial infarction during the acute phase of Kawasaki disease. Coronary Artery Dis 1995; 6:857-64.
20. Crowchuk DP, Kumar M, Vielhaber M.M., et al. Kawasaki disease presenting with thrombocytopenia. Am J Dis Child 1990; 144:19-20.
21. Suntharalingam G, Perry MR, Ward S, et al Cytokine storm in a phase 1 trial of the anti-CD28 monoclonal antibody TGN1412. N Engl J Med 2006; 355:1018-28.
22. Rajasekaran S, Kruse K, Kovey K., et al. Therapeutic role of anakinra, an interleukin-1 receptor antagonist, in the management of secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunction/macrophage activating syndrome in critically ill children. Pediatr Crit Care Med 2014; 15:401-8.