[CIS PIDD] [cis-pidd] lymphohistiocytosis vs ALPS?

[Nacho Gonzalez]

Dear  Wilmer,

It sounds like ALPS syndrome.
vit B12? sFASL?
ALPS can resemble HLH ( http://www.ncbi.nlm.nih.gov/pubmed/24101757)
Did you check for specific DNT cells? with your data the patient has 21%
DNT (ab + gd) which is quite high.

Hope this helps

Best regards

Luis Ignacio Gonzalez-Granado
Immunodeficiencies Unit
Hospital 12 octubre
Madrid. Spain




2015-02-16 11:23 GMT+01:00 Wilmer Cordova Calderon <wilmer.cordova at gmail.com
>:

> Best regards
> In Lima Perú find this case that 2 years ago no find a specific diagnostic
> and child  disease is progressing and complicating other non-immune areas.
> We will be eternally grateful to your help and collaborating diagnosis.
> Thamk you.
>
> Problems:
> 1.        Recurrent pancytopenia associated
> 2.        frequent infections (Pneumonia)
> background:
> physiological
> Psychomotor development 4m, sat at 7-8m, yearling way.
> Current treatment:
> 1. multivitamin Forkids
> 2. Captopril ¼ c / 24h-heart failure secondary portal hypertension.
> Hospitalizations
> 1. 2nd 9m pneumonia and anemia Hb 4g%, thrombocytopenia 5d Huancayo
> 2. 3rd 6m pneumonia and anemia thrombocytopenia hepatosplenomegaly 7d
> Huancayo
> 3. Pneumonia anemia thrombocytopenia 6m 4th cervical lymphadenopathy 5d
> Huancayo
> 4. 5th pneumonia and anemia thrombocytopenia 5d Huancayo Transfusion PG:
> 5. 5th (October 2014) purulent lymphadenopathy, anemia, thrombocytopenia
> CMV, HTP, dilated heart disease and anemia Transfusion 5d PG:
> infections:
> 1. Intestinal infections 3v the first year. furoxone antibiotic.
> 2. SOB 1ª6m.
> Metabolic studies. Liver biopsy with normal results.
> Transfusions: PG 4 chances.
> 02/11/2015
> October 2014 Table 1. Respiratory, painful cervical lymphadenopathy,
> content purulent drainage, PCR for CMV 394 copies / ml (positive> 200),
> pancytopenia (leukocytes 3150, HB 6.6, platelets 107, 000, creat 0.31, FA
> 1423, reticulocytes 4 %) fever, jaundice.
> Eco doppler: HT Portal.
> TEM TAP: hepatosplenomegaly (liver 164mm, 215mm spleen), liquid
> perisplenic, multiple retroperitoneal lymph nodes, mesenteric, iliac and
> inguinal up to 15mm.
> Cervical Doppler ganglion formations with central and peripheral
> vasculature, reagent aspect, in the context of lymphoproliferative aspect.
> Pulmonary moderate to severe dilated cardiomyopathy, pericardial effusion,
> mild systolic dysfunction FE 55% PSAP 62 mmHg HT echocardiography.
> Treatment: Meropenem and vancomycin; Intravenous Ganciclovir, with
> improvement of lymphadenopathy and infectious picture.
> •        Biopsy of cervical and inguinal lymphadenopathy: Lymph node with
> reactive changes predominance of hyperplasia and sinus histiocytosis T zone
> with occasional eritrofagocitosis. Fibrosis; discrete architectural
> distortion. CMV neg, CD20 + lymph B, CD3 + in Lymphoma T, DTT neg, CD10 +
> in follicular centers, Ki67 + cells in replication.
> Nonspecific reactive lymphoid hyperplasia, immunohistochemistry negative
> for lymphoid neoplasia, immunoreaction for LMP1 negative.
> Control abdominal Ecodoppler: higado135mm, spleen 166mm.
> CMV viral load <200 copies / ml
> Dilated cardiomyopathy, mild systolic dysfunction FEvi55% HT moderate
> pulmonary PSAP 47 mmHg
> 02.11.15 Cold symptoms. Lymphadenopathy multiple cervical, axillary and
> inguinal approx 2cm phones.
> Diagnosis
> 1. Primary Immunodeficiency Vs High.
> 2. d / c Lymphohistiocytosis recurrent infectious reactive vs ALPS
> 3. Secondary hypersplenism
> 4. Hypertension Portal
> 5. Pulmonary Hypertension
> 6. dilated cardiomyopathy.
> 3.        CMV infection treated
>
> Marzo 14
> Linfos totales        1630
> Cd4        349        21%
> Cd8        929        57%
> Cd3        1331
> Linfos T cd45 cd3        1331        81
> Cd19        57        3%
> NK                45
> nkt                3.4
> Abril 14
> IgA        168
> IgG        3177
> IgM        282
> IgE        8
>
> --
> *Atte *
> Wilmer Córdova
> *Inmunología Alergología*
> *Instituto Nacional de Salud del Niño - Perú*
> http://www.isn.gob.pe
> wcordova at insn.gob.pe
> facebook.com/wilmer.cordova.71
> twitter: @wilmer_cordova
>
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