Hemophagocytosis in a Severe COVID-19 Patient

The novel coronavirus(SARS-CoV-2)infection has become a heavy burden on global health.Although the coronavirus disease 2019(COVID-19)may adversely affect multiple organs and systems of infected patients,to the best of our knowledge,there is little investigation of the SARS-CoV-2’s impact on bone marrow.Our clinical and cytological findings in this case of severe COVID-19 infection provide novel insights into the pathogenesis of SARS-CoV-2 infection in the hematopoietic system.We recommend that physicians consider SARS-CoV-2 infection’s effect on bone marrow in patients who are slow to recover and suggest that a better understanding of the bone marrow morphology in COVID-19-infected patients is needed.

Typhoid with pancytopenia:Revisiting a forgotten foe:Two case reports

BACKGROUND Typhoid fever is a public health problem in Asia and Africa.Pancytopenia has been rarely reported during the 20th century.Reports during the last 20 years are scarce.CASE SUMMARY Our first patient was a young adult male presenting with febrile neutropenia whose blood and bone marrow cultures grew Salmonella typhi.He recovered before discharge from the hospital.The second was a primigravida who had an abortion following a febrile illness and was found to have pancytopenia.The Widal test showed high initial titers,and she was presumptively treated for typhoid.Convalescence showed a doubling of Widal titers.CONCLUSION Typhoid fever continued to show up as a fever with cytopenia demanding significant effort and time in working up such patients.In developing countries,the liaison with typhoid continues.

Intensive care unit complications and outcomes of adult patients with hemophagocytic lymphohistiocytosis: A retrospective study of 16 cases

AIM To study the management, complications and outcomes of adult patients admitted with hemophagocytic lymphohistiocytosis(HLH) in the intensive care unit(ICU).METHODS We performed a retrospective observational study of adult patients with the diagnosis of "HLH" admitted to the two academic medical ICUs of Baylor College of Medicine between 01/01/2013 to 06/30/2017. HLH was diagnosed using the HLH-2004 criteria proposed by the Histiocyte Society.RESULTS Sixteen adult cases of HLH were admitted to the medical ICUs over 4 years.Median age of presentation was 49 years and 10(63%) were males. Median Sequential Organ Failure Assessment(SOFA) score at the time of ICU admission was 10. Median ICU length of stay(LOS) was 11.5 d and median hospital LOS was 29 d. Septic shock and acute respiratory failure accounted for majority of diagnoses necessitating ICU admission. Septic shock was the most common ICU complication seen in(88%) patients, followed by acute kidney injury(81%) and acute respiratory failure requiring mechanical ventilation(75%). Nine patients(56%) developed disseminated intravascular coagulation and eight(50%) had acute liver failure. 10 episodes of clinically significant bleeding were observed.Multi system organ failure was the most common cause of death seen in 12(75%)patients. The 30 d mortality was 37%(6 cases) and 90 d mortality was 81%(13 cases). There was no difference in mortality based on age(above or less than 50 years), SOFA score on ICU admission(more than or less than 10),immunosuppression, time to diagnose HLH or direct ICU admission versus floor transfer.CONCLUSION HLH is a devastating disease associated with poor outcomes in ICU. Intensivists need to have a high degree of clinical suspicion for HLH in patients with septic shock/multi system organ failure and progressive bi/pancytopenia who are not responding to standard management in ICU.

Hemophagocytic lymphohistiocytosis secondary to composite lymphoma:Two case reports

BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare and life-threatening disease caused by inherited pathogenic mutations and acquired dysregulations of the immune system.Composite lymphoma is defined as two or more morphologically and immunophenotypically distinct lymphomas that occur in a single patient.Here,we report two cases of HLH secondary to composite lymphoma with mixed lineage features of T-and B-cell marker expression both in the bone marrow and lymph nodes in adult patients.CASE SUMMARY Two patients were diagnosed with HLH based on the occurrence of fever,pancytopenia,lymphadenopathy,splenomegaly,hemophagocytosis and hyperferritinemia.Immunohistochemical staining of the axillary lymph node and bone marrow in case 1 showed typical features of combined B-cell and T-cell lymphoma.In addition,a lymph node gene study revealed rearrangement of the T-cell receptor chain and the immunoglobulin gene.Morphology and immunohistochemistry studies of a lymph node biopsy in case 2 showed typical features of T cell lymphoma,but immunophenotyping by flow cytometry analysis of bone marrow aspirate showed B cell lymphoma involvement.The patients were treated with high-dose methylprednisolone combined with etoposide to control aggressive HLH progression.The patients also received immunochemotherapy with the R-CHOP(rituximab,cyclophosphamide,doxorubicin,vincristine,and prednisone)regimen immediately after diagnosis.Both patients presented with highly aggressive lymphoma,and died of severe infection or uncontrolled HLH.CONCLUSION We present two rare cases with overwhelming hemophagocytosis along with composite T-and B-cell lymphoma,which posed a diagnostic dilemma.HLH caused by composite lymphoma was characterized by poor clinical outcomes.

Pregnancy・lnduced Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review

Hemophagocytic lymphohistiocytosis(HLH)is a rare but devastating disease characterized by dysregulated immune response and hyp er inflammation.To our knowledge,pregnancy-induced HLH has been rarely reported in the literature.A 30-year・old pregnant woman presented persistent fever for 21 days since 17 weeks of pregnancy.The possible etiologies such as infection,autoimmune disorder,and malignancy had been ruled out based on a series of exhaustive examinations.The disease progressed despite the use of broadspectrum antibiotics and dexamethasone.The patient was diagnosed as pregnancy-induced HLH,and finally recovered completely after termination of pregnancy by caesarean and the continuous use of glucocorticoid which played a crucial part in controlling hyp er inflammation.Pregnancy-induced HLH could be fatal if effective treatment was not initiated timely.Further studies are needed to improve early diagnosis and etiology identification of HLH.

Successful treatment of adult-onset still disease caused by pulmonary infection-associated hemophagocytic lymphohistiocytosis: A case report

BACKGROUND Adult-onset still disease(AOSD) and hemophagocytic syndrome(HPS) are two inflammatory diseases with very similar clinical manifestations. HPS is one of the most serious complications of AOSD and its risk of death is very high. It is difficult to identify HPS early in patients with AOSD, but early identification and proper treatment directly affects the prognosis.CASE SUMMARY A 39-year-old male showed a high spiking fever and myalgia. Laboratory data revealed elevated white blood cell, serum ferritin, and neutrophil percentage.However, his fever failed to relieve after a clear diagnosis of AOSD caused by pulmonary infection and treatment by antibiotics and corticosteroids;further laboratory data showed elevated serum ferritin, C-reactive protein, erythrocyte sedimentation rate and triglyceride, as well as liver abnormalities. Bone marrow smear showed hemophagocytosis. Secondary HPS was definitely diagnosed. The high fever disappeared and the laboratory findings returned to normal values after treatment by high-dose intravenous methylprednisolone and methotrexate.CONCLUSION For AOSD patients with high suspicion of HPS, active examination needs to be considered for early diagnosis, and timely using of adequate amount of corticosteroids is the key to reducing risk of HPS death.

Unusual spontaneous improvement in Asian variant of intravascular large B-cell lymphoma

The clinical course of the Asian variant of intravascular large B-cell lymphoma (AIVL) is generally very aggressive. We describe a case of AIVL demonstrating an unusual clinical course, with spontaneous improvement. An 81-year-old man with high-grade fever and thrombocytopenia was admitted to our hospital. Although we could not confirm the origin of his symptoms, they disappeared completely without intervention within 2 weeks. Three months later, however, thrombocytopenia reappeared and progressed. Finally, he was readmitted due to a subdural hemorrhage with high fever and he finally died of rapidly progressive multiple organ failure. Autopsy findings revealed the presence of B-cell lymphoma cells in microscopic vessels of many organs as well as hemophagocytosis in the bone marrow. He was diagnosed with AIVL with an unusual indolent clinical course with spontaneous improvement.