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Background Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory condition with uncontrolled activation of lymphocytes and macrophages. Besides a primary (genetic) form, HLH can also be triggered by malignant, autoimmune and infectious diseases. HLH recurrences are rarely described, usually only in primary HLH. Parvovirus B19 (PVB19) Infection is one of the rare and rather benign causes of HLH. Since the infection usually results in long-lasting immunity, recurrent viremia is very uncommon. Case presentation We report an unusual case of a young female with recurrent PVB19 infection that led to repeated episodes of HLH. The first episode occurred at the age of 25 years with a three-week history of high fever and nonspecific accompanying symptoms. The diagnosis of HLH was confirmed by HLH-2004 criteria and HScore, PVB19 viremia was detected as underlying cause. Following guideline-based therapy, the patient was symptom-free for one year, before similar symptoms recurred in a milder form. Again, PVB19 was detected and HLH was diagnosed according to HScore. After successful treatment and a nine-month symptom-free interval, a third phase of hyperinflammation with low PVB19 viremia occurred; this time, treatment with a corticosteroid and intravenous immunoglobulin was initiated before the presence of clear diagnostic criteria for HLH. No further events occurred in the following three years. Conclusions In the case of our patient, the recurrent viremia triggered three episodes of hyperinflammation, two of which were clearly diagnosed as HLH. To our knowledge, this is the first published case of recurrent HLH due to PVB19 infection. Therefore, the case gives new insights in triggering mechanisms for HLH.
Background Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory condition with uncontrolled activation of lymphocytes and macrophages. Besides a primary (genetic) form, HLH can also be triggered by malignant, autoimmune and infectious diseases. HLH recurrences are rarely described, usually only in primary HLH. Parvovirus B19 (PVB19) Infection is one of the rare and rather benign causes of HLH. Since the infection usually results in long-lasting immunity, recurrent viremia is very uncommon. Case presentation We report an unusual case of a young female with recurrent PVB19 infection that led to repeated episodes of HLH. The first episode occurred at the age of 25 years with a three-week history of high fever and nonspecific accompanying symptoms. The diagnosis of HLH was confirmed by HLH-2004 criteria and HScore, PVB19 viremia was detected as underlying cause. Following guideline-based therapy, the patient was symptom-free for one year, before similar symptoms recurred in a milder form. Again, PVB19 was detected and HLH was diagnosed according to HScore. After successful treatment and a nine-month symptom-free interval, a third phase of hyperinflammation with low PVB19 viremia occurred; this time, treatment with a corticosteroid and intravenous immunoglobulin was initiated before the presence of clear diagnostic criteria for HLH. No further events occurred in the following three years. Conclusions In the case of our patient, the recurrent viremia triggered three episodes of hyperinflammation, two of which were clearly diagnosed as HLH. To our knowledge, this is the first published case of recurrent HLH due to PVB19 infection. Therefore, the case gives new insights in triggering mechanisms for HLH.
Introduction and Aim: Haemophagocytic Lymphohistiocytic Syndrome (HLH) is a fatal disease with characteristic proliferation of histiocytes in the organs including bone marrow, liver, spleen, kidney, csf, meninges, lymph nodes and subcutaneous tissue which phagocytes the blood cells. In all unexplained cases of cytopenias, organomegaly and pyrexia of unknown origin the HLH should be thought off. Though there are established criteria to diagnose, early diagnosis can reduce the mortality rate due to HLH. Documenting the haemophagocytes in the biopsy can sometimes be very helpful in arriving at the diagnosis. Materials and Methods: We identified patients who were diagnosed with or suspected to have HLH by bone marrow aspiration or biopsy admitted to Sri Ramachandra Medical College Hospital and Research Institution from January 2010 to June 2017. We retrospectively reviewed patients’ medical records that have haemophagocytes in bone marrow aspirations and collected complete clinical history and laboratory findings. Those history included fever, cytopenias, and organomegaly. Their triglyceride levels, ferritin, ESR or any other abnormal elevated lab values were also noted down. If they had any established diagnosis then that was also noted. Results: This is a descriptive study and the details of the data like clinical history, clinical features, laboratory investigations and abnormal values are represented using tabular column, Pie chart and bar diagrams. Conclusion: From pathological point of view, documenting each case presenting with Haemophagocytes and looking into the factors like age, significant laboratory values along with clinical features can be a valuable tool to come to a provisional diagnosis and might help the clinician to proceed with the treatment for a life-threatening disease without much delay.
An otherwise healthy nine-year-old female who spoke only French presented with abdominal pain, vomiting, intermittent fevers, fatigue, and headache. She then quickly became febrile and altered requiring intubation. When treating a healthy child, the physician may initially develop a differential that includes common illnesses. Yet, as emergency medicine providers, we must be thinking about the “zebras” in order to not miss potentially deadly, curable diseases.
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