Familial and Acquired Hemophagocytic Lymphohistiocytosis (Annual Review of Medicine Book 63)

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The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose. Exam is located at www. Hemophagocytic lymphohistiocytosis HLH is a life-threatening hyperinflammatory syndrome resulting from a highly stimulated but ineffective immune response, which leads to overactivation of macrophages and lymphocytes, hemophagocytosis, severe systemic inflammation, and multiorgan damage.

This syndrome may be the consequence of a genetic defect in cytolytic activity of natural killer NK cells and cytotoxic T lymphocytes or may be promoted by an acquired immunodeficiency. HLH is classified as primary genetic or secondary acquired depending on whether a genetic disorder predisposing to HLH has been identified. Furthermore, HLH can be facilitated by acquired immune deficiencies, iatrogenic immune suppression, and organ or stem cell transplantation. HLH represents the extreme upper end on a gradient of adequate inflammatory response to excessive hyperinflammation; therefore, only the magnitude of clinical and laboratory abnormalities and progressiveness of the symptoms are characteristic of the syndrome.

However, it is always challenging to diagnose an episode of HLH, especially at an early stage when the entire picture of the disease is still incomplete. Furthermore, several underlying conditions such as septicemia 3 or lymphoma 4 can mimic or underlie HLH. Consequently, HLH remains an underrecognized cause of death in patients with septic shock and multiorgan failure.

Nowadays, the diagnostic guidelines for HLH proposed by the Histiocyte Society are still the most widely used criteria to define and diagnose HLH in clinical practice, 6 although these were poorly validated for most cases encountered. This is especially true for acquired forms of HLH and in the adult population, since those guidelines were initially developed to diagnose primary forms of HLH in a pediatric population. Recently, alternative parameters have been evaluated to define a new diagnostic score: However, it has only been validated for the diagnosis of reactive forms of HLH in an adult cohort.

The aim of the present study is to compare the performances of the HLH guidelines with those of the H-score to identify patients with HLH in a retrospective cohort, including both adult and pediatric patients for whom the diagnosis of HLH was suspected in the routine practice of four university hospitals. We retrospectively conducted a keyword search including hemophagocytosis or macrophage activation in the hematology laboratory database of four university-affiliated tertiary hospitals one pediatric center, one oncology adult center, and two major general hospitals in Brussels, Belgium.

The search included all bone marrow aspirates requested between January and December and encompassed the final result field, allowing extraction of cases with evidence of hemophagocytosis and cases in which no evidence of hemophagocytosis had been detected. Only the first aspiration was considered for each patient.

Hemophagocytic lymphohistiocytosis: review of etiologies and management

We then retrospectively reviewed the medical records of these patients. The following laboratory data were also collected: First, we extracted the biological parameters concomitant to bone marrow aspiration in a range of 2 days before or after corresponding to the presentation data set. Second, we recorded the highest or lowest value of each biological parameter registered during the episode corresponding to the diagnostic confirmation data set, allowing the determination of the maximal value of the scores reached for each patient.

The study was approved by the ethics committees of each institution.

Patients were classified as having HLH based on the final diagnosis retained by the medical team caring for the patient. For doubtful cases, two of the authors A. The control group consisted of patients for whom the diagnosis was not retained by the medical team but did not include undetermined cases. As neither NK cell activity nor sCD25 levels were determined in most patients, the HLH diagnostic rule was amended to presume the diagnosis of HLH when at least either four or five among six of the remaining criteria were met.

Materials and Methods

The H-score was determined as described by Fardet et al. Categorical variables are presented as an absolute number expressed in terms of percentage. Comparison of categorical variables was done using the Fisher exact test, and diagnostic odds ratios were calculated for each criterion. To evaluate the diagnostic performances of the HLH guidelines and the H-score to discriminate patients with HLH from control patients, we determined their diagnostic sensitivity, specificity, and accurate classification percentage by taking as a reference the diagnosis retained by the medical team.

For the H-score, we evaluated the cutoff proposed by Fardet et al 8 and an optimal cutoff value obtained by receiver operating characteristic ROC curve analysis. This optimal cutoff value corresponds to the highest value of the sum of sensitivity and specificity. A query performed in our laboratory database identified patients for whom a bone marrow puncture had been requested because of suspected HLH or revealed hemophagocytosis regardless of the indication.

For one child, the diagnosis of HLH could not be formally excluded.


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For six adults, the diagnosis remained undetermined. Since NK cell activity measurement data and sCD25 levels were available for only five of the patients in our cohort, we chose to omit these criteria from our analysis. The characteristics of the study population are shown in Table 2.

The frequency of fulfillment of H-score items is presented in Table 3 determined with the diagnostic confirmation data set.

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In children with HLH, the prevalence of fever, hepatomegaly, splenomegaly, pancytopenia, and increased levels of aspartate aminotransferase was higher than in the control group. Missing data concerned only the control group: Percentage of fulfillment of clinical and biological items in children white bars and adults black bars with hemophagocytic lymphohistiocytosis HLH determined with the diagnostic confirmation data set.

P value determined using the Fisher exact test. Receiver operating characteristic ROC curve analysis for diagnosis of hemophagocytic lymphohistiocytosis in children A , B and adults C , D with H-score computed with the presentation data set A , C or maximal H-score value obtained with the diagnostic confirmation data set B , D. The optimal cutoff was selected as the threshold giving the highest value for the sum of sensitivity and specificity.

In adults at initial presentation, we observed that the best trade-off between sensitivity and specificity was provided by the H-score with an adapted cutoff. With this data set, the best trade-off between sensitivity and specificity was comparable between five of six criteria of the adapted HLH guidelines and H-score.

Discrepant results between the H-score and hemophagocytic lymphohistiocytosis HLH — guidelines observed with the diagnostic confirmation data set: For the H-score, the broken lines indicate the cutoff of Fardet et al, 8 and the solid lines indicate the optimal cutoff.

Hemophagocytic Lymphohistiocytosis: Advocacy Groups and Clinical Trials

Diagnosis of discrepant results for the controls are as follows. A , In adults: B , In children: Seven pediatric controls had an H-score above Figure 3B , of whom four met four of six criteria of the adapted HLH guidelines. The diagnoses retained for these patients were graft vs host disease, thiamine-responsive megaloblastic anemia, systemic Langerhans cell histiocytosis, and progressive familial cholestasis type 2. The baby with an undetermined diagnosis, for whom HLH could not be formally excluded, had an H-score of and met five of the six criteria of the adapted HLH guidelines.

She was a preterm neonate with multisystem organ failure who died 1 week after birth. They included a patient with systemic lupus erythematosus suspected of having either HLH or catastrophic antiphospholipid syndrome who rapidly died after admission and another patient with a posttransplantation reactivation of Epstein-Barr virus. Of the remaining four patients, one had an H-score higher than but less than and met three of the six criteria of the adapted HLH guidelines.

He was admitted for acute myeloid leukemia with sepsis. Two patients had an H-score less than and met three of the six criteria of the adapted HLH guidelines. The first patient had a pulmonary infection treated empirically, and the second patient was admitted for an acute leukemia and died rapidly after admission. The last case had an H-score less than and met five of the six criteria of the adapted HLH guidelines. He was admitted for an acute monoblastic leukemia with pulmonary embolism and disseminated intravascular coagulation.

In this study, we retrospectively assessed the performances of the H-score to diagnose HLH in two distinct cohorts of pediatric and adult patients, and we compared them with the adapted HLH guidelines. The patients were selected on the basis of a bone marrow morphologic examination explicitly reporting the absence or presence of hemophagocytosis. The term hemophagocytosis is also mentioned if hemophagocytosis is incidentally observed regardless of the clinical information received.

Even if the presence of hemophagocytosis has a very low predictive value 9 for the diagnosis of HLH, bone marrow examination is still recommended for HLH suspicion mainly to rule out or identify other confounding or underlying diseases. Our search strategy restricted our evaluation to patients suspected of having HLH who underwent bone marrow aspiration.

Despite the possibility of this selection bias, the different etiologies underlying reactive hemophagocytic syndrome encountered in our population are representative of the heterogeneity of the causes of the disease and in agreement with recently published data for adult cases. Read more Read less. Kindle Cloud Reader Read instantly in your browser. Product details File Size: Annual Reviews December 5, Publication Date: December 5, Sold by: Share your thoughts with other customers.

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Type 2 familial hemophagocytic lymphohistiocytosis in half brothers

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