Table 4. Generalized CSH: summary of... | American Society of Hematology

Table 4.

Generalized CSH: summary of clinical and pathologic features of reported cases

Case/report	Age, y/sex	Presenting symptoms	Diagnosis	Paraprotein	Sites of deposition	Clinical course
Jones 1999¹	50/M	Bone pain	MM	S	BM, LN	10 mo
	66/M	Splenomegaly, cold agglutinin	LPL	S: biclonal U: biclonal	BM, spleen	8 mo
	70/M	Splenomegaly	LPL	ND	BM, LN	Transformed to LCL at 24 mo
	70/M	Abdominal pain, splenomegaly	LPL	S: biclonal	BM, spleen, LN	4 mo
Garcia 1998²	44/M	Transient dyspnea, bilateral proptosis	LPL	S: IgG κ	BM, liver, spleen, LN, and diverse additional sites^4-150	18 mo
Schaefer 1996⁷	75/M	Fatal renal failure, anemia, hyperbilirubinemia	LPL	S: biclonal IgM κ > IgG λ	BM, liver^4-150	DOD
	55/M	Not reported	MM	S: IgG κ	BM, liver	Not reported
Yamamoto 1991¹¹	71/M	Blurred vision	MM	S: IgG κ U: κ	BM, liver, spleen, LN, and diverse additional sites^4-150	4 y
Takahashi 1987¹³	60/M	Fractures of the lumbar vertebrae; bone pain	MM, A^4-150	S: IgA κ U: BJP κ	BM, liver, spleen, LN, and diverse additional sites^4-150	45 mo
Itagaki 1981¹⁷	69/F	NA	MM	S: IgG κ	BM, systemic	NA
Mullen 1981¹⁸	52/M	Low back pain, weight loss	MM	S: IgG κ U: κ	BM, liver, LN, and diverse additional sites^4-150	6.5 y
Terashima 1978²²	58/M	Adult Fanconi syndrome	L-chain storage histiocytosis (MGUS)	S: κ U: κ BJP	BM, liver, spleen, LN, and diverse additional sites^4-150	3 y
Kjeldsberg 1977²³	60/M	Anorexia, fatigue, and pallor	MM, A^4-150	S: κ	BM, liver, spleen^4-150	11 mo
Finkel 1973²⁶	57/F	Adult Fanconi syndrome	KLP, A^4-150	U: κ BJP	BM, liver, abdominal LN, stomach^4-150	10 y
Ito 1970²⁷	53/F	NA	MM	U: κ BJP	BM, liver, spleen, LN, adrenals, kidneys^4-150	5 y
Dedmon 1963²⁹	51/M	Adult Fanconi syndrome	MM	Not reported	BM, LN	10 mo
Engele 1957³⁰	38/M	Adult Fanconi syndrome	MM	U: BJP	BM, spleen	2.5 y
Brass 1948³²	73/F	Not reported	MM	Not reported	BM, liver, spleen^4-150	Not reported
Agress 1940⁵⁶	55/F	NA	RE and PH	NA	BM, liver, spleen, LN, adrenals, kidneys^4-150	1 mo
Ritchie 1936³³	36/F	NA	RE	U: BJP	BM, spleen	1 mo
Glaus 1917³⁴	67/M	Cutaneous elastolysis	MM, A^4-150	U: BJP	BM, LN, skin, testes^4-150	2 y
Present case	73/M	Weight loss, fatigue	MM	S&U: IgA κ	BM, liver, spleen, LN, and diverse additional sites^4-150	7 mo

Case/report	Age, y/sex	Presenting symptoms	Diagnosis	Paraprotein	Sites of deposition	Clinical course
Jones 1999¹	50/M	Bone pain	MM	S	BM, LN	10 mo
	66/M	Splenomegaly, cold agglutinin	LPL	S: biclonal U: biclonal	BM, spleen	8 mo
	70/M	Splenomegaly	LPL	ND	BM, LN	Transformed to LCL at 24 mo
	70/M	Abdominal pain, splenomegaly	LPL	S: biclonal	BM, spleen, LN	4 mo
Garcia 1998²	44/M	Transient dyspnea, bilateral proptosis	LPL	S: IgG κ	BM, liver, spleen, LN, and diverse additional sites^4-150	18 mo
Schaefer 1996⁷	75/M	Fatal renal failure, anemia, hyperbilirubinemia	LPL	S: biclonal IgM κ > IgG λ	BM, liver^4-150	DOD
	55/M	Not reported	MM	S: IgG κ	BM, liver	Not reported
Yamamoto 1991¹¹	71/M	Blurred vision	MM	S: IgG κ U: κ	BM, liver, spleen, LN, and diverse additional sites^4-150	4 y
Takahashi 1987¹³	60/M	Fractures of the lumbar vertebrae; bone pain	MM, A^4-150	S: IgA κ U: BJP κ	BM, liver, spleen, LN, and diverse additional sites^4-150	45 mo
Itagaki 1981¹⁷	69/F	NA	MM	S: IgG κ	BM, systemic	NA
Mullen 1981¹⁸	52/M	Low back pain, weight loss	MM	S: IgG κ U: κ	BM, liver, LN, and diverse additional sites^4-150	6.5 y
Terashima 1978²²	58/M	Adult Fanconi syndrome	L-chain storage histiocytosis (MGUS)	S: κ U: κ BJP	BM, liver, spleen, LN, and diverse additional sites^4-150	3 y
Kjeldsberg 1977²³	60/M	Anorexia, fatigue, and pallor	MM, A^4-150	S: κ	BM, liver, spleen^4-150	11 mo
Finkel 1973²⁶	57/F	Adult Fanconi syndrome	KLP, A^4-150	U: κ BJP	BM, liver, abdominal LN, stomach^4-150	10 y
Ito 1970²⁷	53/F	NA	MM	U: κ BJP	BM, liver, spleen, LN, adrenals, kidneys^4-150	5 y
Dedmon 1963²⁹	51/M	Adult Fanconi syndrome	MM	Not reported	BM, LN	10 mo
Engele 1957³⁰	38/M	Adult Fanconi syndrome	MM	U: BJP	BM, spleen	2.5 y
Brass 1948³²	73/F	Not reported	MM	Not reported	BM, liver, spleen^4-150	Not reported
Agress 1940⁵⁶	55/F	NA	RE and PH	NA	BM, liver, spleen, LN, adrenals, kidneys^4-150	1 mo
Ritchie 1936³³	36/F	NA	RE	U: BJP	BM, spleen	1 mo
Glaus 1917³⁴	67/M	Cutaneous elastolysis	MM, A^4-150	U: BJP	BM, LN, skin, testes^4-150	2 y
Present case	73/M	Weight loss, fatigue	MM	S&U: IgA κ	BM, liver, spleen, LN, and diverse additional sites^4-150	7 mo

S indicates serum M spike; BM, bone marrow; LN, lymph nodes; U, urine M spike; ND, not determined; LCL, large-cell lymphoma; DOD, died of disease; A, amyloidosis; BJP, Bence Jones proteinuria; NA, not available data; KLP, κ light-chain proteinuria; RE, reticuloendotheliosis; PH, purpura hemorrhagica.

F4-150

Autopsy findings.

View Large

This Feature Is Available To Subscribers Only