COAGULATION DISORDERS:

KASABACH-MERRITT SYNDROME

Group 1 BSMT 3D
Paniza, Erika Joy
Villanueva, Andrew Arnold D.
Yandan, Charis
KASABACH-MERRITT SYNDROME
 hemangioma with thrombocytopenia or
hemangioma thrombocytopenia syndrome
 is a rare complication

 Haig Haigouni Kasabach and Katharine Krom

Merritt (1940).

Etymology:
 Hema(blood)+angioma(cancer or tumor)
ETIOLOGY

 Cause is unkown

 A secondary effect due to:

-Vascular Angiomas
-Thrombocytopenia
-Immune reaction
EPIDEMIOLOGY
 a rare disorder
 affects males and females equally

 diagnosis is most often made during infancy but

older children have been reported with this
phenomenon.
 NO HEREDITARY BASIS FOR DIAGNOSIS
PATHOPHYSIOLOGY
IMMUNOLOGIC

 CD34+ vascular progenitor endothelial cell(Civin, Tindle et al)

-seen during earlier years of life
-cells are also found in umbilical cord, bone marrow,
hematopoietic cell, etc.

 CD34
-cluster of differentiation
-cell surface glycoprotein
-cell-cell adhesion factor
CD34+ CELL MOLECULES:
 Junctional Adhesion Molecule(K. Stellos et. al)
-mediates interaction with platelets and
endothelial cells
-adhesion over immobilized platelets.

 Sialomucin P-selectin (CD162) and Integrin Mac-

1 (CD11b-CD18) (Janowska-Wieczorek A, et al. )
-molecules that interact and bind with platelets
PLATELET-PROGENITOR CELL
INTERACTION:
 Platelet functions(while adhered):
 differentiation of progenitor cells

 secrete of stromal cell–derived factor 1 (SDF-1)

-Stem cell chemokine
-recruits circulating progenitor cells and induce
differentiation of the latter into endothelial cells.
HISTOPATHOLOGY:
 Histopathology of KMS(Alvarez-Mendoza A, et al.)

 Tufted Angioma:
-characterized by a proliferation of endothelial cells
positive for CD34

 Kaposiform Hemangioendothelioma:
-luminal endothelial cells were positive for CD34+
HISTOPATHOLOGY:
HISTOPATHOLOGY:
HEMATOLOGIC:
 Thrombocytopenia:
-tumors can trap platelets, causing
severe thrombocytopenia.
-defines KMS together with Hemangioma
HEMATOLOGIC:
 Consumptive coagulopathy:
-use up of clotting factors, such as fibrinogen
-can progress to disseminated intravascular coagulation(DIC) and
even death

 Microangiopathic Hemolytic Anemia:

-secondary to microangiopathic destruction (physical damage) of
the RBCs that results to Schistocytes
-can be expressed as mild, moderate, or severe.
LABORATORY DIAGNOSIS AND FINDINGS
1. Complete blood count (CBC) count with
reticulocyte, platelet count, and peripheral
smear examination
2. Activated Partial Thromboplastin Time
3. Prothrombin Time
4. D-dimer test ( Fragment D-dimer; Fibrin
Degradation Fragment)
5. Ultrasound, MRI or CT scan
- Doppler Flow studies
6. Histopathology/Histology to determine subtypes
of hemangioma
 COMPLETE BLOOD COUNT WITH
RETICULOCYTE, PLATELET COUNT, AND
PERIPHERAL SMEAR EXAMINATION

 Microangiopathic hemolytic anemia

- RBC fragmentation, occurring in
association with small vessel disease.
- Thrombotic lesions in small blood vessels
- Burr cells and schistocytic RBC
 Thrombocytopenia

- Lower than normal number of platelets in

the blood.
BURR CELLS SCHISTOCYTES
ACTIVATED PARTIAL THROMBOPLASTIN
TIME
• Measures the time required to generate thrombin
and fibrin polymers via the intrinsic and common
pathways
• Prolonged aPTT
 PROTHROMBIN TIME
 Generation of thrombin and the formation of
fibrin via the extrinsic and common pathway
- Prolonged in patients with significant
dissegmented intravascular disorder
- Fibrinogen levels are low
- Prominent fibrinogenopenia
- Usually elevated fibrin split (degration)
products
- Depressed clotting factors V and VII
 D-DIMER TEST
 Used to determine if further testing is necessary
to help diagnose diseases and conditions that
cause hypercoagulability
- Low-grade chronic DIC may be present
- D-dimer is elevated in patients with DIC.
 ULTRASOUND, MRI AND CT-SCAN
 MRI or CT commonly reveals a vascular
enhancing mass that is difficult to differentiate
from a vascular malformation
- Doppler flow studies
 HISTOLOGY/HISTOPATHOLOGY
 To determine subtypes of hemangioma
- Kaposiform hemangioendothelioma of
infancy and childhood
- Aggregates of rounded dilated capillaries,
lined by attenuated endothelial cells with small
dark nuclei and filled with red blood cells
- Infiltrative pattern of the cellular areas in
the dermis and subcutaneous fat and muscles,
generally containing few obvious vascular lumina
KAPOSIFORM HEMANGIOENDOTHELIOMA
CLINICAL SIGNS AND SYMPTOMS
 Visible cutaneous giant hemangioma or multiple
smaller hemangiomas
 Enlarged abdomen

 Hepatomegaly or jaundice

 Petechiae, bruising, and frank bleeding

 Painful lesions

 Anemia
 TREATMENT
 No single pharmacologic therapy

AGENTS THAT HAVE BEEN TRIED, WITH

VARYING SUCCESS INCLUDES:
 Corticosteroids
 Interferon alfa
 Aminocaproic acid
 Aspirin
 Heparin
 Vincristine
 Actinomycin D
 Propranolol
NON PHARMACOLOGIC TREATMENT
 Surgical resection
INTERVENTIONAL RADIOLOGIC
PROCEDURES
 OTHER TREATMENT OPTIONS:
 INTERMITTENT PNEUMATIC
COMPRESSION
 RADIATION THERAPY
 REFERENCES:

 Urgent Care Dermatology: Symptom-Based Diagnosis By

James E. Fitzpatrick, Whitney A. High
 Turgeon, Mary Louise, Clinical Hematology Theory and
Procedures,5th edition, page 416
 Rodak's Hematology Clinical Principles and Applications
by Keohone, E.M., et al ( 5th Edition 2016), pages 775-
776
 https://rarediseases.org/rare-diseases/kasabach-merritt-
phenomenon/
 https://labtestsonline.org/understanding/analytes/d-
dimer/tab/test/
 https://www.healthdirect.gov.au/d-dimer-test
 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4088914/