Heavy Chain Disease

Background

This disease is characterized by monoclonal immunoglobulin composed of incomplete heavy chains (IgM, IgG, IgA). No light chains are identified. Unlike multiple myeloma, the bone marrow is sporadically involved.

SYNONYMS None

INCIDENCE Very rare
Alpha chain disease most common

AGE-RANGE AND MEDIAN

Gamma
Median 61 years

Mu
Median 48 years

Alpha
2-3rd decades

PATHOGENESIS CHARACTERIZATION

Monoclonal proliferation of lymphocytes, plasma cell or both

LABORATORY/RADIOLOGIC/OTHER TESTS CHARACTERIZATION

Gamma Serum hypogammaglobulinemia and monoclonal protein with incomplete gamma chain
Urine protein<1 g/24 hours
Anemia 80% of cases
Leukopenia and thrombocytopenia common

Mu 50% of patients produce monoclonal light chains that do not assemble with the heavy chain
Excreted in urine as Bence Jones Proteins

Alpha Alpha chain secreting plasma cells

GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION

Gamma Hepatosplenomegaly and lymphadenopathy in 60%

Mu Usually long history of chronic lymphocytic leukemia
Increased hepatosplenomegaly and rare lymphadenopathy

Alpha 2-3rd decades
Mainly involves the gastrointestinal tract
Hepatosplenomegaly and lymphadenopathy rare

HISTOLOGICAL TYPES CHARACTERIZATION

Gamma Bone marrow involved in 2/3 of patients with plasma cells predominating in 15%

Mu 2/3 of patients have vacuolated plasma cells in bone marrow

Alpha Mainly involves the gastrointestinal tract with heavy infiltration of the lamina propria by plasma cells
Bone marrow usually normal

Atlas of Tumor Pathology-Tumors of the Bone Marrow. Volume 9, Third Series. AFIP Press. 1994.

Commonly Used Terms

Hematologic-Lymphatic Diseases

Multiple myeloma

Last Updated 4/1/2001

SYNONYMS	None
INCIDENCE	Very rare Alpha chain disease most common
AGE-RANGE AND MEDIAN
Gamma	Median 61 years
Mu	Median 48 years
Alpha	2-3rd decades

PATHOGENESIS	CHARACTERIZATION
Monoclonal proliferation of lymphocytes, plasma cell or both

LABORATORY/RADIOLOGIC/OTHER TESTS	CHARACTERIZATION
Gamma	Serum hypogammaglobulinemia and monoclonal protein with incomplete gamma chain Urine protein<1 g/24 hours Anemia 80% of cases Leukopenia and thrombocytopenia common
Mu	50% of patients produce monoclonal light chains that do not assemble with the heavy chain Excreted in urine as Bence Jones Proteins
Alpha	Alpha chain secreting plasma cells

GROSS APPEARANCE/CLINICAL VARIANTS	CHARACTERIZATION
Gamma	Hepatosplenomegaly and lymphadenopathy in 60%
Mu	Usually long history of chronic lymphocytic leukemia Increased hepatosplenomegaly and rare lymphadenopathy
Alpha	2-3rd decades Mainly involves the gastrointestinal tract Hepatosplenomegaly and lymphadenopathy rare

HISTOLOGICAL TYPES	CHARACTERIZATION
Gamma	Bone marrow involved in 2/3 of patients with plasma cells predominating in 15%
Mu	2/3 of patients have vacuolated plasma cells in bone marrow
Alpha	Mainly involves the gastrointestinal tract with heavy infiltration of the lamina propria by plasma cells Bone marrow usually normal