MAHAS and DIC Detailed Overview

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Questions and Answers

Which condition is characterized by generalized activation of the coagulation and fibrinolytic systems?

  • Thrombotic Thrombocytopenic Purpura (TTP)
  • Disseminated Intravascular Coagulation (DIC) (correct)
  • Hemolytic Uremic Syndrome (HUS)
  • Hellp Syndrome

Which type of red blood cell is characterized by a crescent shape and long projections?

  • Helmet cell
  • Target cell
  • Drepanoctye (Irreversible Sickle Cell) (correct)
  • Oat-shaped cell

What is a common association for target cells?

  • Acute promyelocytic leukemia
  • Thrombotic Thrombocytopenic Purpura
  • Liver disease (correct)
  • Acute infections

Which of the following statements is true regarding MAHA disorders?

<p>They involve RBC fragmentation due to small vessel obstruction. (B)</p> Signup and view all the answers

Which test is NOT typically associated with diagnosing DIC?

<p>Complete blood count (CBC) (B)</p> Signup and view all the answers

Flashcards

MAHA

A group of disorders causing RBC fragmentation and thrombocytopenia, typically from small blood vessel narrowing due to fibrin or platelets.

TTP

A type of MAHA, causing microvascular thrombi formation.

HELLP

A pregnancy complication involving hemolysis, elevated liver enzymes, and low platelet count; another type of MAHA.

HUS

A type of MAHA, often triggered by infections, causing kidney damage.

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DIC

A consequence, not a disease, characterized by abnormal clotting and bleeding due to widespread coagulation activation.

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Helmet cell

A specific type of fragmented red blood cell, shaped like a helmet, often seen in MAHA.

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Drepanocyte

Sickled red blood cell, also called meniscocyte, due to abnormal hemoglobin.

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Irreversible Sickle Cell

A form of drepanocyte with permanent crescent shape, with long projections, and fragmentation upon reoxygenation.

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Target Cell

Red blood cell with a central stained area and a thin outer hemoglobin rim.

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Codocyte

Red blood cell with an increased cell membrane-to-volume ratio, bell-shaped under electron microscopy, also known as target cell.

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Liver Disease

One cause of target cells.

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Hemoglobinopathy

One cause of target cells.

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Thalassemia

One cause of target cells.

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Study Notes

MAHAS

  • Group of disorders characterized by RBC fragmentation and thrombocytopenia
  • Major types include TTP, HELLP, HUS, and DIC
  • Characterized by narrowing/obstruction of small blood vessels by fibrin/platelet aggregates
  • Helmet cell is a helmet-shaped schistocyte that can appear in MAHA

Additional information about DIC

  • Also known as defibrination syndrome or consumption coagulopathy
  • Generalized over-activation of coagulation and fibrinolytic systems
  • Not a disease, but a consequence
  • Some tests include D-dimer and PF 1+2
  • Related conditions: tissue trauma, obstetric complications, mucus-secreting tumors, acute infections (e.g., malaria, Gram-negative septicemia), and acute promyelocytic leukemia

DREPANOCYTE

  • Also known as a sickle cell or meniscocyte
  • Drepan = sickle
  • RBCs
  • Two forms of drepanocytes:
    • Irreversible Sickle Cell:
      • Crescent-shaped
      • Long projections
      • Fragment upon reoxygenation
    • Oat-shaped cell:
      • Less pronounced projections
      • Return to biconcave disk shape upon reoxygenation

TARGET CELL

  • Also known as leptocyte/codocyte
  • Platycyte, or Bull's eye cell
  • Mexican hat cell or Greek helmet cell
  • RBCs with centrally stained area and thin outer rim of hemoglobin
  • Related conditions include liver disease, certain hemoglobinopathies (like thalassemia), hereditary leptocytosis, Mediterranean anemia, and LCAT (lecithin cholesterol acyltransferase) deficiency
  • Codocytes are RBCs with an increased cell membrane-to-volume ratio
  • Appear bell-shaped on electron microscopy

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