Asian Hematology Research Journal

Routine Diagnostic Immunophenotypic Profiling in AML Identifies Chemo Resistant Patients and Predicts Treatment Outcomes

Birva Raiya — 2024-09-27

Aim: This study aimed to examine different phenotypic stages of arrest observed in Acute Myeloid Leukemia.

Materials and Methods: A total of 220 patients with Acute Myeloid Leukemia (AML) were categorized into five subgroups Hematopoietic stem cells (HSCs), Multipotent Progenitors (MPPs), Common Myeloid Progenitors (CMPs), Common Myeloid Progenitors (CMPs), Granulocyte/Macrophage Progenitors (GMPs) based on their pattern of stage of leukemia arrest (SLA) using an Immunophenotypic profile with routine myeloid markers (MPO, CD13, CD33, CD14, HLADR, and CD34) at diagnosis and were correlated with clinicohematological parameters, molecular and cytogenetic characteristics, and disease status.

Results: The distribution of the SLA phenotypes showed 21% with MPP, 26% with CMP, 21% with GMP, and 37% with GP/MP and none of the patient showed HCP phenotype. In relation to clinicohematological parameters, male patients had maturation arrest at an earlier stage of differentiation (MPP/CMP), whereas female showed in a later stage (GP/MP). High white blood cell (WBC) and blast counts exhibited maturation arrest at the later stage (GP/MP. In relation with FAB subtypes, 83% AML-M3, 42% of AML-M4, and 50% of AML-M5 are associated with the GP/MP phenotype, while 67% of AML-M0 and 23% of AML-M1 are associated with the MPP phenotype. Further, maturation arrest at the MPP level was associated with aberrant CD7 expression. In relation with mutations in FLT3-ITD/ FLT3 D835 were associated with MPP (27%) and CMP (40%), C-kit mutations with CMP stage (67%), whereas NPM1 mutations (75%) and CEBPA mutations (50%) were showed arrest at the progenitor stage (GP/MP). In relation to treatment response, 75% with the MPP phenotype, 13% with CMP, 27% with GMP, and 19% with GP/MP showed persistent leukemic activity. This suggests that the MPP group is the most resistant to chemotherapy.

Conclusion: Acute Myeloid Leukemia Immunophenotyping can identify a new SLA phenotype that effectively correlates with leukemic cell behavior and helps predict major genetic subgroups at diagnosis, along with their response to standard intensive chemotherapy.

Salmonella Paratyphoid Induced Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review

Kainat Bibi — 2024-10-15

Background: Haemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome characterized by an excessive inflammatory response. Limited data exist on adult HLH.

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a condition in which immune hyperactivation and deregulation causes hemophagocytosis and organ disorder due to activated histiocytes and macrophages. Symptoms include fever, splenomegaly, abdominal distension and hepatomegaly.

Case Report: We report the case of a forty year old man who presented to our Hospital with fever and loose stools and upon investigation had a pancytopenia with hypertriglyceridemia, hepatosplenomegaly, these clinical and laboratory findings raised suspicion of HLH secondary to typhoid fever. The patient was treated with immunosuppression (dexamethasone) and antibiotics and showed remarkable recovery. Hemophagocytic lymphohistiocytosis should be suspected in patients with tropical infections like enteric fever, tuberculosis, malaria, dengue, etc. that worsen despite appropriate treatment, as late diagnosis is associated with greater mortality.

In this national, retrospective cohort study, we analysed data from the Pub Med database collected between October 1, 2006 and December 31, 2023 in making of this literature review.

Results and Conclusion: Typhoid fever caused by the bacterium salmonella typhi and paratyphi is a serious febrile illness and is more common in the developing countries As a first line intervention, treating the etiology of HLH would be an efficient way to restrict the disease's progression. We report a rare and unique case of secondary HLH due to typhoid fever, this complication is often missed and a prompt diagnosis is essential for the treatment of this fatal disease. It can be primary (familial) or secondary (acquired). Clinically diagnoses can be difficult because of similarities in signs and symptoms with other illnesses as it generally presents with fever, loose stools, and decreased appetite and on Investigation one can expect pan or bi cytopenias, hepatosplenomegaly, hypertriglyceridemia, hypofibrinogenemia. Timely diagnosis of HLH makes it very treatable in majority of cases.

Flow Cytometric Analysis of Immunophenotypic Characteristics of B-cell Lymphoproliferative Disorders: A Review

Balasuriya B L T — 2024-09-11

Mature B cell neoplasm present a diverse group of disorders characterized by distinct clinical presentations, pathological features, and outcomes. These neoplasms originate from B-Cell lineages, exhibiting clonality along with morphological, immunophenotypic, and genetic characteristics. This review provides a detailed evaluation of global variations, diagnostic challenges, and prognostic significance of B cell non-Hodgkin lymphomas (B-NHL). It emphasizes the critical role of immunophenotypic markers, such as CD5, and CD10, and the application of advanced diagnostic technologies like flow cytometry in guiding personalized treatment strategies.

The literature search was conducted using key terms like “B cell lymphoproliferative disorders”,” BCLPDs”, “B-NHL” and “Flow cytometric immunophenotyping of B-CLPDs” across multiple databases and google scholar.

The review highlights the key findings from worldwide studies, offering insights into how these markers and technologies contribute to improved diagnostic accuracy and patient outcomes.

Additionally, it discusses the implications of aberrant marker expression and bi-clonality, which further complicate diagnosis but hold significant prognostic value. This comprehensive analysis aims to assist clinicians and researches in refining diagnostic criteria and optimizing therapeutic approaches for B-NHL globally.