Abstract
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.
Highlights
Introduction and AimHaemophagocytic 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
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
The tests like soluble CD25 levels and NK cell activity were not done in our institution, we considered the haemophagocytes presence in the bone marrow aspiration and biopsy along with that other criterion like fever, splenomegaly, cytopenias, triglyceride levels elevated ferritin levels and other abnormal lab values including LDH, Abnormal LFT and elevated ESR levels
Summary
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. Syndrome (HLH) is a fatal disease leading to end organ damage and death. There is characteristic proliferation of histiocytes in the organs including bone marrow, liver, spleen, kidney, meninges, lymph nodes and subcutaneous tissue which phagocytes the blood cells [1,2] Earlier HLH is classified as primary form and secondary form, the former is genetically mediated and they have abnormal cytotoxic function of NK cell and T cells and the latter form is associated with infections and malignancy. The basic mechanism is hypercytokinemia which results in end organ damage which in turn leads to death [1,3]. In all unexplained cases of cytopenias, organomegaly and pyrexia of unknown origin the HLH should be thought off
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