Systemic Mastocytosis

Back of a patient showing diffuse reddish-brown maculopapular lesions consistent with Cutaneous Mastocytosis or Urticaria Pigmentosa.

Systemic Mastocytosis (SM): Symptoms, Diagnosis, and Management: Systemic Mastocytosis (SM) is a rare, complex hematologic neoplasm characterized by the uncontrolled accumulation of atypical mast cells in various internal tissues and organs, most commonly the bone marrow, spleen, liver, and GI tract. This chronic condition primarily affects adults. Because signs and symptoms, such as urticaria pigmentosa and anaphylaxis, vary widely based […]

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Antiphospholipid Syndrome

Lupus Anticoagulant

Antiphospholipid Syndrome (APS), also known as Hughes Syndrome, is an autoimmune disorder characterized by the presence of antiphospholipid antibodies (aPL) that promote abnormal blood clot formation (thrombosis) in both arterial and venous circulation. APS may occur as a primary condition or develop secondary to other autoimmune diseases, most commonly systemic lupus erythematosus (SLE). Clinically, patients may present with deep vein […]

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Tumor Immunology

Cancer Cells

Tumor immunology is the study of how the immune system interacts with cancer cells, focusing on the mechanisms by which tumors develop, evade immune detection, and resist destruction. Understanding the tumor microenvironment, immune checkpoints, and tumor-associated antigens is essential in both hematology and oncology, as these insights drive the development of advanced immunotherapies such as checkpoint inhibitors, CAR-T cell therapy, […]

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Plasmapheresis

Plasmapheresis

What is Plasmapheresis? Plasmapheresis, also known as therapeutic plasma exchange (TPE), is a medical procedure that involves removing a patient’s plasma and replacing it with a substitute such as saline, albumin, or donor plasma. The process separates plasma from blood cells using a centrifuge or membrane filtration system, allowing the selective removal of pathogenic antibodies, immune complexes, and toxins. Plasmapheresis […]

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Hemophagocytosis

Hemophagocytosis

Introduction: Hemophagocytic Lymphohistiocytosis (HLH), also known as hemophagocytosis, is a rare but life-threatening hyperinflammatory syndrome caused by uncontrolled activation of the immune system. It results from defects in natural killer (NK) cell and cytotoxic T-cell regulation, leading to excessive cytokine release and accumulation of activated macrophages and lymphocytes in multiple organs. These overactive immune cells can attack the body’s own […]

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Checkpoint Inhibitors

Activated T cell binding to a tumor cell illustrating how checkpoint inhibitors enhance immune recognition in cancer treatment.

A key function of the immune system is its ability to distinguish healthy self-cells from harmful “foreign” cells, ensuring precise immune surveillance and protection. Cancer cells can evade this process by exploiting immune-checkpoint pathways, allowing tumors to grow unchecked. Checkpoint inhibitors work by blocking these pathways and restoring the immune system’s ability to recognize and attack cancer. Immune system uses “checkpoints” […]

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Cold Agglutinin Disease

cold-agglutinin-disease

Cold Agglutinin Disease (CAD) is a rare autoimmune hemolytic anemia (AIHA) caused by the presence of cold-reactive autoantibodies (cold agglutinins) that target red blood cells at temperatures below 30°C (86°F). These autoantibodies lead to red blood cell agglutination, complement activation, and subsequent immune-mediated hemolysis, especially in peripheral body areas exposed to cold. CAD accounts for approximately 15% of all autoimmune […]

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Cryoglobulinemia

Lower-leg vasculitis and purpuric skin changes caused by cryoglobulinemia.

Cryoglobulins are immunoglobulins—either monoclonal or mixed—that precipitate at low temperatures and resolubilize on warming, forming the basis of cryoglobulinemia. Although trace amounts may occur in some healthy individuals, clinically relevant cryoglobulinemia is typically associated with abnormal immunoglobulin production seen in autoimmune diseases, lymphoproliferative disorders, and chronic infections such as hepatitis C. When precipitated, cryoglobulins can increase blood viscosity and obstruct […]

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