Clinical features of CD
| . | UCD . | iMCD-NOS . | iMCD-TAFRO . | POEMS-associated MCD . | HHV8-MCD . |
|---|---|---|---|---|---|
| Age | Fourth decade | Fifth to sixth decade | Fifth decade | Fifth decade | Fifth decade (HIV+); |
| seventh decade (HIV−) | |||||
| Systemic symptoms* | ± | ++ | +++ | ++ | +++ |
| None or compressive | And occasional PN | And anasarca | And Kaposi sarcoma | ||
| Lymphadenopathy | Central most common; often bulky | Peripheral plus central; often small volume | Peripheral plus central; often small volume | Peripheral plus central | Peripheral plus central; often small volume |
| Organomegaly | ± | ++ | +++ | +++ | +++ |
| Abnormal inflammatory markers† | ± | +++ | +++ | ++ | +++ |
| Also increased procalcitonin | |||||
| Anemia, thrombocytopenia, abnormal LFTs | ± | ++ | +++ | ± | +++ |
| Sometimes thrombocytosis | HHV-8 DNA detectable in plasma | ||||
| Hypergammaglobulinemia | ± | +++ | ± | +, small M-spike | +++ |
| Renal dysfunction | − | + | ++ | + | ++ |
| Intravascular coagulation and fibrinolysis | |||||
| Autoimmune phenomena | Rare, but PNP can be seen | ++ | ± | ± | Positive DAT in 46%; MG in 28% |
| AIHA, PNP, ITP, interstitial lung disease | |||||
| Pathologic features | Usually HV variant | Usually PC variant | Usually mixed or hypervascular type | Usually mixed or PC type | Usually PC variant and often plasmablastic |
| Therapy | Surgery | IL-6–targeted therapy; rituximab; systemic therapies | Same as iMCD, but also calcineurin inhibitors | Radiation (localized) | Rituximab, etoposide |
| Myeloma-type therapy including ASCT (disseminated) | |||||
| Clinical course | Benign | Variable | Very aggressive | Aggressive | Aggressive |
| Risk for lymphoma | + | + | ± | ± | ++ |
| . | UCD . | iMCD-NOS . | iMCD-TAFRO . | POEMS-associated MCD . | HHV8-MCD . |
|---|---|---|---|---|---|
| Age | Fourth decade | Fifth to sixth decade | Fifth decade | Fifth decade | Fifth decade (HIV+); |
| seventh decade (HIV−) | |||||
| Systemic symptoms* | ± | ++ | +++ | ++ | +++ |
| None or compressive | And occasional PN | And anasarca | And Kaposi sarcoma | ||
| Lymphadenopathy | Central most common; often bulky | Peripheral plus central; often small volume | Peripheral plus central; often small volume | Peripheral plus central | Peripheral plus central; often small volume |
| Organomegaly | ± | ++ | +++ | +++ | +++ |
| Abnormal inflammatory markers† | ± | +++ | +++ | ++ | +++ |
| Also increased procalcitonin | |||||
| Anemia, thrombocytopenia, abnormal LFTs | ± | ++ | +++ | ± | +++ |
| Sometimes thrombocytosis | HHV-8 DNA detectable in plasma | ||||
| Hypergammaglobulinemia | ± | +++ | ± | +, small M-spike | +++ |
| Renal dysfunction | − | + | ++ | + | ++ |
| Intravascular coagulation and fibrinolysis | |||||
| Autoimmune phenomena | Rare, but PNP can be seen | ++ | ± | ± | Positive DAT in 46%; MG in 28% |
| AIHA, PNP, ITP, interstitial lung disease | |||||
| Pathologic features | Usually HV variant | Usually PC variant | Usually mixed or hypervascular type | Usually mixed or PC type | Usually PC variant and often plasmablastic |
| Therapy | Surgery | IL-6–targeted therapy; rituximab; systemic therapies | Same as iMCD, but also calcineurin inhibitors | Radiation (localized) | Rituximab, etoposide |
| Myeloma-type therapy including ASCT (disseminated) | |||||
| Clinical course | Benign | Variable | Very aggressive | Aggressive | Aggressive |
| Risk for lymphoma | + | + | ± | ± | ++ |
Data compiled from Weisenburger et al,6 Iwaki et al,10 Chronowski et al,13 Oksenhendler et al,17 Frizzera et al,54 Oksenhendler et al,56 Menke et al,57 Fujimoto et al,64 and Nishimura et al.65
+, sometimes present; ++, often present; +++, very often present; ±, rarely present; AIHA, autoimmune hemolytic anemia; ASCT, autologous stem cell transplant; DAT, direct antiglobulin test; ITP, immune thrombocytopenic purpura; LFT, liver function test; MG, monoclonal gammopathy; PN, peripheral neuropathy; PNP, paraneoplastic pemphigus.
Fever, sweats, weight loss, malaise, effusions, autoimmune, and respiratory symptoms.
Increased ESR, CRP, cholinesterase, ferritin, and low albumin.